Dr. Foxley

Caitlin Foxley, MD, followed a nontraditional path to medicine. While attending Colorado State University in Fort Collins, she took a course in economics to fill her schedule. She enjoyed economics so much, she majored in it.

One year into a graduate program, however, she decided the business world wasn’t for her. She left school and went to work for the American Heart Association to do fundraising and health education, and there she found her calling.

Inspired by the association’s emphasis on disease prevention and the passion displayed by the physician volunteers, she decided medicine could be a good fit for her. She began taking courses part time each semester for a couple of years until she fulfilled all of her prerequisites, then applied to medical school.

Today, Dr. Foxley is medical director of Inpatient Management Inc.’s hospitalist program at Nebraska Medical Center Hospitals, a 680-bed tertiary-care center and Level 1 trauma center in Omaha. “I’d always liked science when I was younger, and it was always a strong point for me,” she says. “But working with the American Heart Association is really what sparked my interest in medicine.”

Question: How did your work with the AHA guide you into medicine?

Answer: I liked the message of prevention of diseases. Even then, in the late 1980s, they were looking at evidence-based medicine. It made sense to me. It seemed like a good way to make a difference. I thought, “This is something I could do and enjoy.”

Q: When did you make the change?

A: I had to do a few undergrad prerequisites, since economics didn’t really prepare me for medical school. I took one class per semester for a couple years while working for the heart association, then applied to medical school.

Q: After medical school, you spent five years in traditional internal-medicine practice. Did you consider going directly into hospital medicine?

A: When I got out of residency (in 2001), there really weren’t many opportunities for hospitalists in Nebraska. It was something I knew I’d like, but it wasn’t available.

Q: You made the switch in 2006. What prompted the move?

A: I was really frustrated doing traditional outpatient medicine. It was becoming increasingly difficult to provide the quality of care I wanted to give my patients. I was seeing people with complex medical problems, and I was having to do it in 15-minute increments.

Somebody I knew from the medical community had formed a hospitalist group, so I started working with him. It’s been a great career move, and it’s something I really love.

Q: What did you enjoy most about the hospital setting?

A: Everything. I like the challenge of seeing patients who are more complicated than those in the office. If I want to spend a half-hour or hour with a patient, I have that opportunity. I like the immediacy of the results, and I like being able to talk to consultants in the hospital to help formulate a diagnosis and a plan. All of that provides for better care.

Q: You assumed your current position in 2008. Did you always envision yourself moving into a leadership role?

A: I never really thought about it, but I’m glad I made the switch. There are days when it’s not all fun and games, but it’s very much been a learning opportunity. I’ve enjoyed it, and it has helped me become a better physician.

Q: How so?

A: I see the big picture. I can see what the administration wants, and I have an inside view to what hospital leadership thinks we can do better. I can share that with the other doctors. It helps us deliver better care knowing what the goals are for the hospital, our group, and the patients.

Q: What is your biggest challenge?

A: Having to be the “bad guy” in an administrative role.

Q: Have you learned any techniques that make that process easier?

A: It’s important, especially when you have to deal with conflict, to be open-minded and listen carefully to all sides of the situation. You have to give everyone a chance to speak their piece.

Q: You recently completed a Leukemia and Lymphoma Society Century Ride. What was that experience like?

A: It was like nothing I’d ever done before. I liked getting on my bike and riding a few miles, but I never thought I’d be able to ride 100 miles in one day. It was a life-changing experience, and I raised over $4,000 for the Leukemia and Lymphoma Society, which felt wonderful.

Q: Did you learn anything that you can apply as a physician?

A: I learned that if you really put your mind to it, you can accomplish a lot. At times, when I’d be going up a difficult hill, I’d think, “This is really hard, but it’s nothing like being the parent of a kid with leukemia.”

Now, as I look at people who are suffering and sick, I remember that. No matter how hard it is for me, I’m not facing what they’re facing. TH

Mark Leiser is a freelance writer based in New Jersey.

Dr. Foxley

Caitlin Foxley, MD, followed a nontraditional path to medicine. While attending Colorado State University in Fort Collins, she took a course in economics to fill her schedule. She enjoyed economics so much, she majored in it.

One year into a graduate program, however, she decided the business world wasn’t for her. She left school and went to work for the American Heart Association to do fundraising and health education, and there she found her calling.

Inspired by the association’s emphasis on disease prevention and the passion displayed by the physician volunteers, she decided medicine could be a good fit for her. She began taking courses part time each semester for a couple of years until she fulfilled all of her prerequisites, then applied to medical school.

Today, Dr. Foxley is medical director of Inpatient Management Inc.’s hospitalist program at Nebraska Medical Center Hospitals, a 680-bed tertiary-care center and Level 1 trauma center in Omaha. “I’d always liked science when I was younger, and it was always a strong point for me,” she says. “But working with the American Heart Association is really what sparked my interest in medicine.”

Question: How did your work with the AHA guide you into medicine?

Answer: I liked the message of prevention of diseases. Even then, in the late 1980s, they were looking at evidence-based medicine. It made sense to me. It seemed like a good way to make a difference. I thought, “This is something I could do and enjoy.”

Q: When did you make the change?

A: I had to do a few undergrad prerequisites, since economics didn’t really prepare me for medical school. I took one class per semester for a couple years while working for the heart association, then applied to medical school.

Q: After medical school, you spent five years in traditional internal-medicine practice. Did you consider going directly into hospital medicine?

A: When I got out of residency (in 2001), there really weren’t many opportunities for hospitalists in Nebraska. It was something I knew I’d like, but it wasn’t available.

Q: You made the switch in 2006. What prompted the move?

A: I was really frustrated doing traditional outpatient medicine. It was becoming increasingly difficult to provide the quality of care I wanted to give my patients. I was seeing people with complex medical problems, and I was having to do it in 15-minute increments.

Somebody I knew from the medical community had formed a hospitalist group, so I started working with him. It’s been a great career move, and it’s something I really love.

Q: What did you enjoy most about the hospital setting?

A: Everything. I like the challenge of seeing patients who are more complicated than those in the office. If I want to spend a half-hour or hour with a patient, I have that opportunity. I like the immediacy of the results, and I like being able to talk to consultants in the hospital to help formulate a diagnosis and a plan. All of that provides for better care.

Q: You assumed your current position in 2008. Did you always envision yourself moving into a leadership role?

A: I never really thought about it, but I’m glad I made the switch. There are days when it’s not all fun and games, but it’s very much been a learning opportunity. I’ve enjoyed it, and it has helped me become a better physician.

Q: How so?

A: I see the big picture. I can see what the administration wants, and I have an inside view to what hospital leadership thinks we can do better. I can share that with the other doctors. It helps us deliver better care knowing what the goals are for the hospital, our group, and the patients.

Q: What is your biggest challenge?

A: Having to be the “bad guy” in an administrative role.

Q: Have you learned any techniques that make that process easier?

A: It’s important, especially when you have to deal with conflict, to be open-minded and listen carefully to all sides of the situation. You have to give everyone a chance to speak their piece.

Q: You recently completed a Leukemia and Lymphoma Society Century Ride. What was that experience like?

A: It was like nothing I’d ever done before. I liked getting on my bike and riding a few miles, but I never thought I’d be able to ride 100 miles in one day. It was a life-changing experience, and I raised over $4,000 for the Leukemia and Lymphoma Society, which felt wonderful.

Q: Did you learn anything that you can apply as a physician?

A: I learned that if you really put your mind to it, you can accomplish a lot. At times, when I’d be going up a difficult hill, I’d think, “This is really hard, but it’s nothing like being the parent of a kid with leukemia.”

Now, as I look at people who are suffering and sick, I remember that. No matter how hard it is for me, I’m not facing what they’re facing. TH

Mark Leiser is a freelance writer based in New Jersey.

Dr. Foxley

Caitlin Foxley, MD, followed a nontraditional path to medicine. While attending Colorado State University in Fort Collins, she took a course in economics to fill her schedule. She enjoyed economics so much, she majored in it.

One year into a graduate program, however, she decided the business world wasn’t for her. She left school and went to work for the American Heart Association to do fundraising and health education, and there she found her calling.

Inspired by the association’s emphasis on disease prevention and the passion displayed by the physician volunteers, she decided medicine could be a good fit for her. She began taking courses part time each semester for a couple of years until she fulfilled all of her prerequisites, then applied to medical school.

Today, Dr. Foxley is medical director of Inpatient Management Inc.’s hospitalist program at Nebraska Medical Center Hospitals, a 680-bed tertiary-care center and Level 1 trauma center in Omaha. “I’d always liked science when I was younger, and it was always a strong point for me,” she says. “But working with the American Heart Association is really what sparked my interest in medicine.”

Question: How did your work with the AHA guide you into medicine?

Answer: I liked the message of prevention of diseases. Even then, in the late 1980s, they were looking at evidence-based medicine. It made sense to me. It seemed like a good way to make a difference. I thought, “This is something I could do and enjoy.”

Q: When did you make the change?

A: I had to do a few undergrad prerequisites, since economics didn’t really prepare me for medical school. I took one class per semester for a couple years while working for the heart association, then applied to medical school.

Q: After medical school, you spent five years in traditional internal-medicine practice. Did you consider going directly into hospital medicine?

A: When I got out of residency (in 2001), there really weren’t many opportunities for hospitalists in Nebraska. It was something I knew I’d like, but it wasn’t available.

Q: You made the switch in 2006. What prompted the move?

A: I was really frustrated doing traditional outpatient medicine. It was becoming increasingly difficult to provide the quality of care I wanted to give my patients. I was seeing people with complex medical problems, and I was having to do it in 15-minute increments.

Somebody I knew from the medical community had formed a hospitalist group, so I started working with him. It’s been a great career move, and it’s something I really love.

Q: What did you enjoy most about the hospital setting?

A: Everything. I like the challenge of seeing patients who are more complicated than those in the office. If I want to spend a half-hour or hour with a patient, I have that opportunity. I like the immediacy of the results, and I like being able to talk to consultants in the hospital to help formulate a diagnosis and a plan. All of that provides for better care.

Q: You assumed your current position in 2008. Did you always envision yourself moving into a leadership role?

A: I never really thought about it, but I’m glad I made the switch. There are days when it’s not all fun and games, but it’s very much been a learning opportunity. I’ve enjoyed it, and it has helped me become a better physician.

Q: How so?

A: I see the big picture. I can see what the administration wants, and I have an inside view to what hospital leadership thinks we can do better. I can share that with the other doctors. It helps us deliver better care knowing what the goals are for the hospital, our group, and the patients.

Q: What is your biggest challenge?

A: Having to be the “bad guy” in an administrative role.

Q: Have you learned any techniques that make that process easier?

A: It’s important, especially when you have to deal with conflict, to be open-minded and listen carefully to all sides of the situation. You have to give everyone a chance to speak their piece.

Q: You recently completed a Leukemia and Lymphoma Society Century Ride. What was that experience like?

A: It was like nothing I’d ever done before. I liked getting on my bike and riding a few miles, but I never thought I’d be able to ride 100 miles in one day. It was a life-changing experience, and I raised over $4,000 for the Leukemia and Lymphoma Society, which felt wonderful.

Q: Did you learn anything that you can apply as a physician?

A: I learned that if you really put your mind to it, you can accomplish a lot. At times, when I’d be going up a difficult hill, I’d think, “This is really hard, but it’s nothing like being the parent of a kid with leukemia.”

Now, as I look at people who are suffering and sick, I remember that. No matter how hard it is for me, I’m not facing what they’re facing. TH

Mark Leiser is a freelance writer based in New Jersey.

It seems like yesterday that I began the journey of being SHM’s president. And now I find myself in the “remains of the day,” reflecting upon what was accomplished, and what remains to be done. Here, then, in the twilight of my day as president, are a few reflections from the most recent chapter in HM before I say goodbye.

The Organizational Chassis

This was the year that the contracts for both the CEO and our journal editor came due. I am pleased to have re-signed our CEO to a three-year contract, ensuring continued leadership of our initiatives during a time in which we cannot pause. I am also pleased that the search committee, with great diligence, has arrived upon a candidate who I am sure will continue the imprimatur of excellence that has defined the Journal of Hospital Medicine during Mark Williams’ tenure. In concert with this appointment is a new contract with JHM’s publisher, Wiley-Blackwell, the terms of which will ensure the continued and expanding impact that the journal has upon HM practice.

I am also pleased with SHM’s huge step forward with respect to the policies and procedures regarding transparency and its external relationships with industry. My column in the December 2010 issue (see “The Story of Us,” p. 43) outlines the progress; the accompanying letter to the editor outlines what remains to be done. The policy revisions are a remarkable step forward in ensuring the integrity of the organization, but I do agree we can do more.

Membership

SHM’s membership now exceeds 12,000, an impressive accomplishment eclipsed only by 88% membership retention. SHM, like no other organization, has built an infrastructure of empowerment, particularly with respect to advancing the goals of quality and patient safety, and people are voting with their feet by joining and sustaining membership with the organization.

Equally impressive is the organization’s ability to maintain the “big tent” as a part of this membership growth. I am pleased with the board’s decision to pass by-laws reforms to ensure that any unique constituency, with sufficient size, would have a provision for placing a representative on the Board of Directors. But even so, the seams of the “big tent” will be increasingly stressed as we continue to grow. Making the adaptations necessary to maintain this “big tent” must remain a priority for the organization.

To address this challenge, three new committees were established within the “membership cluster” this year. The Young Physicians Task Force was divided into two new committees: one committee (Pipeline) was moved to the academic cluster to focus upon our relationships with the educational infrastructure (medical schools and residency training programs) and ensure that hospitalists of the future are better prepared to assume the role, particularly with respect to advancing quality and patient safety.

The second committee from this division, Early Career Hospitalists, remained within membership to ensure that the unique needs of the hospitalist in their first five years of practice were being addressed. My vision from the outset was that there would come a day that a “virtual mentor system” would be in place for the young SHM member, and based upon the work of this committee, I believe the foundation has been laid to realize this dream sooner than you might think.

Does SHM’s leadership reflect the gender, race, religion, ethnicity, and sexual-preference diversity of the 30,000-plus hospitalists practicing in the U.S.? I don’t know the answer, but I suspect that whatever measure of diversity we have reflected in our organizational leadership, it could be better. I am very pleased with the initiation of the Diversity Task Force, which will ensure that SHM is developing leaders from these constituencies within its committees such that, in the coming years, there is meaningful integration of these diversities into the SHM leadership.

If SHM is to fulfill its destiny of changing American healthcare, it will do so only as a part of collaboration with other national organizations and entities. What comes next is too big for one physician organization to enact alone. One such organization is the Veterans Administration healthcare system, and I am pleased that we have started this journey by establishing the VA Affairs Committee. I look forward to seeing what will come of SHM’s collaboration with the VA. I believe that in 10 years’ time, both entities will count themselves fortunate for having engaged in this collaborative journey together.

Academics

From the outset, SHM’s attention to academics was about the pipeline, for despite our diverse practice patterns, we all share one common denominator in that we are where we are today as a product of our training. Much has been said about whether there will be a sufficient number of students and residents entering the practice of HM. But the question is about quality, not quantity. For HM to be sustainable, the best and brightest of our medical students and residents must select HM as a lifelong career, not as a year between residency and subspecialty fellowship. Career decisions are based upon mentors and role models, and the only solution is to ensure that our students and residents are regularly interacting with hospitalists as role models in their medical schools and residencies.

This was the second year for the Academic Hospitalist Academy: an initiative critical to ensuring that the hospitalists with whom our students and residents interact have the educational and leadership skills to be effective as role models. The work by the Academic Practice and Promotion Committee will soon yield a position paper that will establish the benchmarks for hospitalist promotions, empowering chairs and promotion committees to sustain hospitalists within the academic infrastructure. This is the second year of funding young investigators in HM, and SHM’s inclusion in the GEMSTAR program will enable further funding to ensure that the specialty is creating new knowledge.

And, as noted above, the new Pipeline Committee already has been effective in establishing a relationship with the Alliance for Academic Internal Medicine (professors, clerkship directors, residency directors). The collaborative venture, the Quality and Safety Educators Academy (QSEA), will come to fruition early in 2012, further integrating hospitalists as mentors in the educational infrastructure.

And even as you read this, I will be representing SHM in a joint collaborative with AAIM, ABIM, SGIM, and ACP regarding the “milestones project” as a new model of establishing resident competency, ensuring that the knowledge and skills requisite for being a hospitalist will be acquired in residency training in years to come.

Practice Management

After 48 trips over the course of the year, I can tell you that despite how far we have come as a profession, there remains remarkable heterogeneity as to how hospitalist groups are structured. And yet there are common principles that underlie the high-performing teams, principles captured in the work of the Practice Management and Practice Analysis committees. This valuable SHM service as the clearinghouse of best practices must continue to grow.

My guess as to what comes next in the practice of HM is the progressive blurring of the artificial barriers among the ED, the wards, and the ICU. The reality is that hospitalists are increasingly involved in all three domains, providing emergency and critical care as much as they are standard ward management. I am pleased that we are now engaged in discussions with the American College of Emergency Physicians and the Society for Critical Care Medicine, looking for potential areas of collaboration in building the hospital of the future.

Quality and Patient Safety

In the past year, SHM’s mentored-implementation initiatives have continued to expand, now improving more than 100 clinical sites. In their own right, these initiatives are impressive. But the most impressive element is the philosophy that one cookie-cutter strategy is unlikely to work for all systems. Tailoring the strategy to the unique features of the system, under the guidance of a mentor/coach, is the brilliance that has defined SHM’s efforts. Further, it espouses the greater philosophical principle that we are our brother’s keepers.

For meaningful healthcare reform to come to fruition, quality improvement in isolation (i.e. a few ACOs here or there) will be insufficient, a point I made at the White House briefing on healthcare reform. It is the role of a physician society such as SHM to bring together the community of all hospital systems, removed from the mindset of competition, to ensure that what meaningful improvements are made in one system are replicated in others.

SHM has made the jump to the next level in advancing quality by securing resources for a full-time physician quality leader within the organization. The announcement of who this leader will be will follow shortly, though I am pleased that SHM’s commitment to quality and patient safety continues to expand.

But with quality today addressed, what do we do about tomorrow? How do we ensure that those physicians who will follow us (i.e. our current medical students and residents) are better prepared to enact meaningful quality and patient safety as a part of their careers? I am pleased with the work enacted by the Quality Education Committee, establishing a Web-based portal that will serve as the foundation for teaching medical students and residents the essential principles of quality and patient safety.

But meaningful learning requires a “coach,” an educator trained in the principles of teaching and applying these critical skills. To meet this need, SHM has joined forces with the Alliance for Academic Internal Medicine (AAIM) to develop a Quality and Safety Educators Academy, which will take place early in 2012. This academy will train hospitalists interested in teaching quality and patient safety to medical students and residents, using the product developed by the QIE committee as its substrate. The ancillary benefit, of course, is the integration of more hospitalists into the educational infrastructure, exposing students and residents to their potential mentors such that HM becomes a valued career in their minds.

HM’s persistent challenge is the harsh reality that not all hospitalists engage in quality and patient safety. To this end, I am pleased that SHM’s quality database, SQUINT, has come to fruition. While nascent in its development, this Web-based platform will enable those who have enacted quality initiatives to upload their project to a searchable database, further enabling other hospitalists interested in starting a QI project to quickly search for projects that are similar to their hospital’s size, structure, and needs.

As the format for SQUINT will replicate the structure of ABIM’s Practice Improvement Module format, it will provide the added service of empowering hospitalists engaged in Maintenance of Certification (MOC) in Focused Practice in Hospital Medicine. And vice versa, it will enable all who have completed ABIM PIMs to post their QI projects on SQUINT, further leveraging the size and depth of the SQUINT database.

Education

October 2010 marked the first MOC examination with the Focused Practice in Hospital Medicine designation. I am pleased that SHM has not yielded in its efforts to ensure that MOC in HM is not just a piece of paper, but also a tangible process that leverages improved performance on the part of the hospitalist. To assist hospitalists in meeting these requirements, SHM has worked on three medical knowledge modules this year, one that already qualifies for MOC credit and two more expected to be available by this time next year.

The consistent quality of SHM’s educational programming has continued throughout the year. Undoubtedly, many of you will be reading this en route to another exceptional annual meeting in Dallas. Though you will not see this at HM11, the foundation plan for a completely electronic meeting, enabling real-time dialogue between speakers and audience members (via smartphones, etc.), has been set in motion. The fully electronic annual meeting is not far away.

Advocacy

Heading into this past year, the board made the decision to double the resources for the advocacy cluster. SHM has become a major voice in the conversation of healthcare reform, and the advocacy leadership of the organization has been invited to weigh in on all components of the Patient Protection and Affordable Care Act.

From bundling to ACOs, from value-based purchasing to readmissions, I am proud of the message espoused by SHM’s advocacy leadership (www.hospital medicine.org/advocacy). Proud, because the modus operandi that has gained us great credibility among legislators has continued: a message that advocates for the needs of the hospitalist but never at the expense of what is best for the patient.

Of all of the decisions made in the past year, there is none wiser than to have invested in our advocacy infrastructure. The conversation in which we are now involved transcends what is best for the hospitalist—it is a conversation about changing a decades-old healthcare system to something better. And the complexities of this conversation require erudite and wise thought leaders, people who care about the right things.

Going forward, the road will be no less challenging. Walking the line of preserving our specialty while doing what is best for the patient must remain our priority.

A year ago today, I set forth 10 goals:

• Ensure a solid leadership base for the years to come;
• Move the organization to an even higher level of integrity and transparency;
• Augment the “pipeline” of the profession, ensuring that those who come next will be better prepared than we were;
• Augment the infrastructure to advance diversity within the organization;
• Ensure that the philosophy of the “big tent” vision is sustained;
• Ensure that our advocacy efforts are about doing the right thing: providing the safest and highest-quality care for all patients;
• Establish relationships with other organizations;
• Establish an infrastructure that enables all hospitalists to participate in quality and patient-safety initiatives;
• Further establish HM as its own specialty, a specialty known for being the vanguard of quality and patient safety; and
• Ensure that the leaders of the organization who follow me inherit an organization that is better than when I found it.

Only time will tell whether I was successful in meeting these goals, but to the extent we succeeded, I give full credit to the SHM staff, leadership, and member volunteers who made it happen. To the extent that we fell short, I take full responsibility.

It has been an honor to be your president. As with all things in life, success or failure is measured in 10 minutes—those solitary 10 minutes each night before you fall asleep. For it is in those 10 minutes that you find yourself utterly and completely alone with your life; what you said, and what you meant to say; what you did, and what you didn’t do. Despite the ups and downs of the year, I’ve never once begrudged those 10 minutes, for I have nothing but pride in my heart as I think about you and SHM, an organization and a community that is, and will continue to be, one that cares about the right things in life.

So this is me signing off. I look forward to serving HM in whatever way I can as the years transpire.

For now, I look forward to president-elect Joseph Ming Wah Li, MD, SFHM, FACP, continuing this journey. And so should you. I am confident that even better days are soon to come under his leadership. TH

Dr. Wiese is president of SHM.

It seems like yesterday that I began the journey of being SHM’s president. And now I find myself in the “remains of the day,” reflecting upon what was accomplished, and what remains to be done. Here, then, in the twilight of my day as president, are a few reflections from the most recent chapter in HM before I say goodbye.

The Organizational Chassis

This was the year that the contracts for both the CEO and our journal editor came due. I am pleased to have re-signed our CEO to a three-year contract, ensuring continued leadership of our initiatives during a time in which we cannot pause. I am also pleased that the search committee, with great diligence, has arrived upon a candidate who I am sure will continue the imprimatur of excellence that has defined the Journal of Hospital Medicine during Mark Williams’ tenure. In concert with this appointment is a new contract with JHM’s publisher, Wiley-Blackwell, the terms of which will ensure the continued and expanding impact that the journal has upon HM practice.

I am also pleased with SHM’s huge step forward with respect to the policies and procedures regarding transparency and its external relationships with industry. My column in the December 2010 issue (see “The Story of Us,” p. 43) outlines the progress; the accompanying letter to the editor outlines what remains to be done. The policy revisions are a remarkable step forward in ensuring the integrity of the organization, but I do agree we can do more.

Membership

SHM’s membership now exceeds 12,000, an impressive accomplishment eclipsed only by 88% membership retention. SHM, like no other organization, has built an infrastructure of empowerment, particularly with respect to advancing the goals of quality and patient safety, and people are voting with their feet by joining and sustaining membership with the organization.

Equally impressive is the organization’s ability to maintain the “big tent” as a part of this membership growth. I am pleased with the board’s decision to pass by-laws reforms to ensure that any unique constituency, with sufficient size, would have a provision for placing a representative on the Board of Directors. But even so, the seams of the “big tent” will be increasingly stressed as we continue to grow. Making the adaptations necessary to maintain this “big tent” must remain a priority for the organization.

To address this challenge, three new committees were established within the “membership cluster” this year. The Young Physicians Task Force was divided into two new committees: one committee (Pipeline) was moved to the academic cluster to focus upon our relationships with the educational infrastructure (medical schools and residency training programs) and ensure that hospitalists of the future are better prepared to assume the role, particularly with respect to advancing quality and patient safety.

The second committee from this division, Early Career Hospitalists, remained within membership to ensure that the unique needs of the hospitalist in their first five years of practice were being addressed. My vision from the outset was that there would come a day that a “virtual mentor system” would be in place for the young SHM member, and based upon the work of this committee, I believe the foundation has been laid to realize this dream sooner than you might think.

Does SHM’s leadership reflect the gender, race, religion, ethnicity, and sexual-preference diversity of the 30,000-plus hospitalists practicing in the U.S.? I don’t know the answer, but I suspect that whatever measure of diversity we have reflected in our organizational leadership, it could be better. I am very pleased with the initiation of the Diversity Task Force, which will ensure that SHM is developing leaders from these constituencies within its committees such that, in the coming years, there is meaningful integration of these diversities into the SHM leadership.

If SHM is to fulfill its destiny of changing American healthcare, it will do so only as a part of collaboration with other national organizations and entities. What comes next is too big for one physician organization to enact alone. One such organization is the Veterans Administration healthcare system, and I am pleased that we have started this journey by establishing the VA Affairs Committee. I look forward to seeing what will come of SHM’s collaboration with the VA. I believe that in 10 years’ time, both entities will count themselves fortunate for having engaged in this collaborative journey together.

Academics

From the outset, SHM’s attention to academics was about the pipeline, for despite our diverse practice patterns, we all share one common denominator in that we are where we are today as a product of our training. Much has been said about whether there will be a sufficient number of students and residents entering the practice of HM. But the question is about quality, not quantity. For HM to be sustainable, the best and brightest of our medical students and residents must select HM as a lifelong career, not as a year between residency and subspecialty fellowship. Career decisions are based upon mentors and role models, and the only solution is to ensure that our students and residents are regularly interacting with hospitalists as role models in their medical schools and residencies.

This was the second year for the Academic Hospitalist Academy: an initiative critical to ensuring that the hospitalists with whom our students and residents interact have the educational and leadership skills to be effective as role models. The work by the Academic Practice and Promotion Committee will soon yield a position paper that will establish the benchmarks for hospitalist promotions, empowering chairs and promotion committees to sustain hospitalists within the academic infrastructure. This is the second year of funding young investigators in HM, and SHM’s inclusion in the GEMSTAR program will enable further funding to ensure that the specialty is creating new knowledge.

And, as noted above, the new Pipeline Committee already has been effective in establishing a relationship with the Alliance for Academic Internal Medicine (professors, clerkship directors, residency directors). The collaborative venture, the Quality and Safety Educators Academy (QSEA), will come to fruition early in 2012, further integrating hospitalists as mentors in the educational infrastructure.

And even as you read this, I will be representing SHM in a joint collaborative with AAIM, ABIM, SGIM, and ACP regarding the “milestones project” as a new model of establishing resident competency, ensuring that the knowledge and skills requisite for being a hospitalist will be acquired in residency training in years to come.

Practice Management

After 48 trips over the course of the year, I can tell you that despite how far we have come as a profession, there remains remarkable heterogeneity as to how hospitalist groups are structured. And yet there are common principles that underlie the high-performing teams, principles captured in the work of the Practice Management and Practice Analysis committees. This valuable SHM service as the clearinghouse of best practices must continue to grow.

My guess as to what comes next in the practice of HM is the progressive blurring of the artificial barriers among the ED, the wards, and the ICU. The reality is that hospitalists are increasingly involved in all three domains, providing emergency and critical care as much as they are standard ward management. I am pleased that we are now engaged in discussions with the American College of Emergency Physicians and the Society for Critical Care Medicine, looking for potential areas of collaboration in building the hospital of the future.

Quality and Patient Safety

In the past year, SHM’s mentored-implementation initiatives have continued to expand, now improving more than 100 clinical sites. In their own right, these initiatives are impressive. But the most impressive element is the philosophy that one cookie-cutter strategy is unlikely to work for all systems. Tailoring the strategy to the unique features of the system, under the guidance of a mentor/coach, is the brilliance that has defined SHM’s efforts. Further, it espouses the greater philosophical principle that we are our brother’s keepers.

For meaningful healthcare reform to come to fruition, quality improvement in isolation (i.e. a few ACOs here or there) will be insufficient, a point I made at the White House briefing on healthcare reform. It is the role of a physician society such as SHM to bring together the community of all hospital systems, removed from the mindset of competition, to ensure that what meaningful improvements are made in one system are replicated in others.

SHM has made the jump to the next level in advancing quality by securing resources for a full-time physician quality leader within the organization. The announcement of who this leader will be will follow shortly, though I am pleased that SHM’s commitment to quality and patient safety continues to expand.

But with quality today addressed, what do we do about tomorrow? How do we ensure that those physicians who will follow us (i.e. our current medical students and residents) are better prepared to enact meaningful quality and patient safety as a part of their careers? I am pleased with the work enacted by the Quality Education Committee, establishing a Web-based portal that will serve as the foundation for teaching medical students and residents the essential principles of quality and patient safety.

But meaningful learning requires a “coach,” an educator trained in the principles of teaching and applying these critical skills. To meet this need, SHM has joined forces with the Alliance for Academic Internal Medicine (AAIM) to develop a Quality and Safety Educators Academy, which will take place early in 2012. This academy will train hospitalists interested in teaching quality and patient safety to medical students and residents, using the product developed by the QIE committee as its substrate. The ancillary benefit, of course, is the integration of more hospitalists into the educational infrastructure, exposing students and residents to their potential mentors such that HM becomes a valued career in their minds.

HM’s persistent challenge is the harsh reality that not all hospitalists engage in quality and patient safety. To this end, I am pleased that SHM’s quality database, SQUINT, has come to fruition. While nascent in its development, this Web-based platform will enable those who have enacted quality initiatives to upload their project to a searchable database, further enabling other hospitalists interested in starting a QI project to quickly search for projects that are similar to their hospital’s size, structure, and needs.

As the format for SQUINT will replicate the structure of ABIM’s Practice Improvement Module format, it will provide the added service of empowering hospitalists engaged in Maintenance of Certification (MOC) in Focused Practice in Hospital Medicine. And vice versa, it will enable all who have completed ABIM PIMs to post their QI projects on SQUINT, further leveraging the size and depth of the SQUINT database.

Education

October 2010 marked the first MOC examination with the Focused Practice in Hospital Medicine designation. I am pleased that SHM has not yielded in its efforts to ensure that MOC in HM is not just a piece of paper, but also a tangible process that leverages improved performance on the part of the hospitalist. To assist hospitalists in meeting these requirements, SHM has worked on three medical knowledge modules this year, one that already qualifies for MOC credit and two more expected to be available by this time next year.

The consistent quality of SHM’s educational programming has continued throughout the year. Undoubtedly, many of you will be reading this en route to another exceptional annual meeting in Dallas. Though you will not see this at HM11, the foundation plan for a completely electronic meeting, enabling real-time dialogue between speakers and audience members (via smartphones, etc.), has been set in motion. The fully electronic annual meeting is not far away.

Advocacy

Heading into this past year, the board made the decision to double the resources for the advocacy cluster. SHM has become a major voice in the conversation of healthcare reform, and the advocacy leadership of the organization has been invited to weigh in on all components of the Patient Protection and Affordable Care Act.

From bundling to ACOs, from value-based purchasing to readmissions, I am proud of the message espoused by SHM’s advocacy leadership (www.hospital medicine.org/advocacy). Proud, because the modus operandi that has gained us great credibility among legislators has continued: a message that advocates for the needs of the hospitalist but never at the expense of what is best for the patient.

Of all of the decisions made in the past year, there is none wiser than to have invested in our advocacy infrastructure. The conversation in which we are now involved transcends what is best for the hospitalist—it is a conversation about changing a decades-old healthcare system to something better. And the complexities of this conversation require erudite and wise thought leaders, people who care about the right things.

Going forward, the road will be no less challenging. Walking the line of preserving our specialty while doing what is best for the patient must remain our priority.

A year ago today, I set forth 10 goals:

• Ensure a solid leadership base for the years to come;
• Move the organization to an even higher level of integrity and transparency;
• Augment the “pipeline” of the profession, ensuring that those who come next will be better prepared than we were;
• Augment the infrastructure to advance diversity within the organization;
• Ensure that the philosophy of the “big tent” vision is sustained;
• Ensure that our advocacy efforts are about doing the right thing: providing the safest and highest-quality care for all patients;
• Establish relationships with other organizations;
• Establish an infrastructure that enables all hospitalists to participate in quality and patient-safety initiatives;
• Further establish HM as its own specialty, a specialty known for being the vanguard of quality and patient safety; and
• Ensure that the leaders of the organization who follow me inherit an organization that is better than when I found it.

Only time will tell whether I was successful in meeting these goals, but to the extent we succeeded, I give full credit to the SHM staff, leadership, and member volunteers who made it happen. To the extent that we fell short, I take full responsibility.

It has been an honor to be your president. As with all things in life, success or failure is measured in 10 minutes—those solitary 10 minutes each night before you fall asleep. For it is in those 10 minutes that you find yourself utterly and completely alone with your life; what you said, and what you meant to say; what you did, and what you didn’t do. Despite the ups and downs of the year, I’ve never once begrudged those 10 minutes, for I have nothing but pride in my heart as I think about you and SHM, an organization and a community that is, and will continue to be, one that cares about the right things in life.

So this is me signing off. I look forward to serving HM in whatever way I can as the years transpire.

For now, I look forward to president-elect Joseph Ming Wah Li, MD, SFHM, FACP, continuing this journey. And so should you. I am confident that even better days are soon to come under his leadership. TH

Dr. Wiese is president of SHM.

It seems like yesterday that I began the journey of being SHM’s president. And now I find myself in the “remains of the day,” reflecting upon what was accomplished, and what remains to be done. Here, then, in the twilight of my day as president, are a few reflections from the most recent chapter in HM before I say goodbye.

The Organizational Chassis

This was the year that the contracts for both the CEO and our journal editor came due. I am pleased to have re-signed our CEO to a three-year contract, ensuring continued leadership of our initiatives during a time in which we cannot pause. I am also pleased that the search committee, with great diligence, has arrived upon a candidate who I am sure will continue the imprimatur of excellence that has defined the Journal of Hospital Medicine during Mark Williams’ tenure. In concert with this appointment is a new contract with JHM’s publisher, Wiley-Blackwell, the terms of which will ensure the continued and expanding impact that the journal has upon HM practice.

I am also pleased with SHM’s huge step forward with respect to the policies and procedures regarding transparency and its external relationships with industry. My column in the December 2010 issue (see “The Story of Us,” p. 43) outlines the progress; the accompanying letter to the editor outlines what remains to be done. The policy revisions are a remarkable step forward in ensuring the integrity of the organization, but I do agree we can do more.

Membership

SHM’s membership now exceeds 12,000, an impressive accomplishment eclipsed only by 88% membership retention. SHM, like no other organization, has built an infrastructure of empowerment, particularly with respect to advancing the goals of quality and patient safety, and people are voting with their feet by joining and sustaining membership with the organization.

Equally impressive is the organization’s ability to maintain the “big tent” as a part of this membership growth. I am pleased with the board’s decision to pass by-laws reforms to ensure that any unique constituency, with sufficient size, would have a provision for placing a representative on the Board of Directors. But even so, the seams of the “big tent” will be increasingly stressed as we continue to grow. Making the adaptations necessary to maintain this “big tent” must remain a priority for the organization.

To address this challenge, three new committees were established within the “membership cluster” this year. The Young Physicians Task Force was divided into two new committees: one committee (Pipeline) was moved to the academic cluster to focus upon our relationships with the educational infrastructure (medical schools and residency training programs) and ensure that hospitalists of the future are better prepared to assume the role, particularly with respect to advancing quality and patient safety.

The second committee from this division, Early Career Hospitalists, remained within membership to ensure that the unique needs of the hospitalist in their first five years of practice were being addressed. My vision from the outset was that there would come a day that a “virtual mentor system” would be in place for the young SHM member, and based upon the work of this committee, I believe the foundation has been laid to realize this dream sooner than you might think.

Does SHM’s leadership reflect the gender, race, religion, ethnicity, and sexual-preference diversity of the 30,000-plus hospitalists practicing in the U.S.? I don’t know the answer, but I suspect that whatever measure of diversity we have reflected in our organizational leadership, it could be better. I am very pleased with the initiation of the Diversity Task Force, which will ensure that SHM is developing leaders from these constituencies within its committees such that, in the coming years, there is meaningful integration of these diversities into the SHM leadership.

If SHM is to fulfill its destiny of changing American healthcare, it will do so only as a part of collaboration with other national organizations and entities. What comes next is too big for one physician organization to enact alone. One such organization is the Veterans Administration healthcare system, and I am pleased that we have started this journey by establishing the VA Affairs Committee. I look forward to seeing what will come of SHM’s collaboration with the VA. I believe that in 10 years’ time, both entities will count themselves fortunate for having engaged in this collaborative journey together.

Academics

From the outset, SHM’s attention to academics was about the pipeline, for despite our diverse practice patterns, we all share one common denominator in that we are where we are today as a product of our training. Much has been said about whether there will be a sufficient number of students and residents entering the practice of HM. But the question is about quality, not quantity. For HM to be sustainable, the best and brightest of our medical students and residents must select HM as a lifelong career, not as a year between residency and subspecialty fellowship. Career decisions are based upon mentors and role models, and the only solution is to ensure that our students and residents are regularly interacting with hospitalists as role models in their medical schools and residencies.

This was the second year for the Academic Hospitalist Academy: an initiative critical to ensuring that the hospitalists with whom our students and residents interact have the educational and leadership skills to be effective as role models. The work by the Academic Practice and Promotion Committee will soon yield a position paper that will establish the benchmarks for hospitalist promotions, empowering chairs and promotion committees to sustain hospitalists within the academic infrastructure. This is the second year of funding young investigators in HM, and SHM’s inclusion in the GEMSTAR program will enable further funding to ensure that the specialty is creating new knowledge.

And, as noted above, the new Pipeline Committee already has been effective in establishing a relationship with the Alliance for Academic Internal Medicine (professors, clerkship directors, residency directors). The collaborative venture, the Quality and Safety Educators Academy (QSEA), will come to fruition early in 2012, further integrating hospitalists as mentors in the educational infrastructure.

And even as you read this, I will be representing SHM in a joint collaborative with AAIM, ABIM, SGIM, and ACP regarding the “milestones project” as a new model of establishing resident competency, ensuring that the knowledge and skills requisite for being a hospitalist will be acquired in residency training in years to come.

Practice Management

After 48 trips over the course of the year, I can tell you that despite how far we have come as a profession, there remains remarkable heterogeneity as to how hospitalist groups are structured. And yet there are common principles that underlie the high-performing teams, principles captured in the work of the Practice Management and Practice Analysis committees. This valuable SHM service as the clearinghouse of best practices must continue to grow.

My guess as to what comes next in the practice of HM is the progressive blurring of the artificial barriers among the ED, the wards, and the ICU. The reality is that hospitalists are increasingly involved in all three domains, providing emergency and critical care as much as they are standard ward management. I am pleased that we are now engaged in discussions with the American College of Emergency Physicians and the Society for Critical Care Medicine, looking for potential areas of collaboration in building the hospital of the future.

Quality and Patient Safety

In the past year, SHM’s mentored-implementation initiatives have continued to expand, now improving more than 100 clinical sites. In their own right, these initiatives are impressive. But the most impressive element is the philosophy that one cookie-cutter strategy is unlikely to work for all systems. Tailoring the strategy to the unique features of the system, under the guidance of a mentor/coach, is the brilliance that has defined SHM’s efforts. Further, it espouses the greater philosophical principle that we are our brother’s keepers.

For meaningful healthcare reform to come to fruition, quality improvement in isolation (i.e. a few ACOs here or there) will be insufficient, a point I made at the White House briefing on healthcare reform. It is the role of a physician society such as SHM to bring together the community of all hospital systems, removed from the mindset of competition, to ensure that what meaningful improvements are made in one system are replicated in others.

SHM has made the jump to the next level in advancing quality by securing resources for a full-time physician quality leader within the organization. The announcement of who this leader will be will follow shortly, though I am pleased that SHM’s commitment to quality and patient safety continues to expand.

But with quality today addressed, what do we do about tomorrow? How do we ensure that those physicians who will follow us (i.e. our current medical students and residents) are better prepared to enact meaningful quality and patient safety as a part of their careers? I am pleased with the work enacted by the Quality Education Committee, establishing a Web-based portal that will serve as the foundation for teaching medical students and residents the essential principles of quality and patient safety.

But meaningful learning requires a “coach,” an educator trained in the principles of teaching and applying these critical skills. To meet this need, SHM has joined forces with the Alliance for Academic Internal Medicine (AAIM) to develop a Quality and Safety Educators Academy, which will take place early in 2012. This academy will train hospitalists interested in teaching quality and patient safety to medical students and residents, using the product developed by the QIE committee as its substrate. The ancillary benefit, of course, is the integration of more hospitalists into the educational infrastructure, exposing students and residents to their potential mentors such that HM becomes a valued career in their minds.

HM’s persistent challenge is the harsh reality that not all hospitalists engage in quality and patient safety. To this end, I am pleased that SHM’s quality database, SQUINT, has come to fruition. While nascent in its development, this Web-based platform will enable those who have enacted quality initiatives to upload their project to a searchable database, further enabling other hospitalists interested in starting a QI project to quickly search for projects that are similar to their hospital’s size, structure, and needs.

As the format for SQUINT will replicate the structure of ABIM’s Practice Improvement Module format, it will provide the added service of empowering hospitalists engaged in Maintenance of Certification (MOC) in Focused Practice in Hospital Medicine. And vice versa, it will enable all who have completed ABIM PIMs to post their QI projects on SQUINT, further leveraging the size and depth of the SQUINT database.

Education

October 2010 marked the first MOC examination with the Focused Practice in Hospital Medicine designation. I am pleased that SHM has not yielded in its efforts to ensure that MOC in HM is not just a piece of paper, but also a tangible process that leverages improved performance on the part of the hospitalist. To assist hospitalists in meeting these requirements, SHM has worked on three medical knowledge modules this year, one that already qualifies for MOC credit and two more expected to be available by this time next year.

The consistent quality of SHM’s educational programming has continued throughout the year. Undoubtedly, many of you will be reading this en route to another exceptional annual meeting in Dallas. Though you will not see this at HM11, the foundation plan for a completely electronic meeting, enabling real-time dialogue between speakers and audience members (via smartphones, etc.), has been set in motion. The fully electronic annual meeting is not far away.

Advocacy

Heading into this past year, the board made the decision to double the resources for the advocacy cluster. SHM has become a major voice in the conversation of healthcare reform, and the advocacy leadership of the organization has been invited to weigh in on all components of the Patient Protection and Affordable Care Act.

From bundling to ACOs, from value-based purchasing to readmissions, I am proud of the message espoused by SHM’s advocacy leadership (www.hospital medicine.org/advocacy). Proud, because the modus operandi that has gained us great credibility among legislators has continued: a message that advocates for the needs of the hospitalist but never at the expense of what is best for the patient.

Of all of the decisions made in the past year, there is none wiser than to have invested in our advocacy infrastructure. The conversation in which we are now involved transcends what is best for the hospitalist—it is a conversation about changing a decades-old healthcare system to something better. And the complexities of this conversation require erudite and wise thought leaders, people who care about the right things.

Going forward, the road will be no less challenging. Walking the line of preserving our specialty while doing what is best for the patient must remain our priority.

A year ago today, I set forth 10 goals:

• Ensure a solid leadership base for the years to come;
• Move the organization to an even higher level of integrity and transparency;
• Augment the “pipeline” of the profession, ensuring that those who come next will be better prepared than we were;
• Augment the infrastructure to advance diversity within the organization;
• Ensure that the philosophy of the “big tent” vision is sustained;
• Ensure that our advocacy efforts are about doing the right thing: providing the safest and highest-quality care for all patients;
• Establish relationships with other organizations;
• Establish an infrastructure that enables all hospitalists to participate in quality and patient-safety initiatives;
• Further establish HM as its own specialty, a specialty known for being the vanguard of quality and patient safety; and
• Ensure that the leaders of the organization who follow me inherit an organization that is better than when I found it.

Only time will tell whether I was successful in meeting these goals, but to the extent we succeeded, I give full credit to the SHM staff, leadership, and member volunteers who made it happen. To the extent that we fell short, I take full responsibility.

It has been an honor to be your president. As with all things in life, success or failure is measured in 10 minutes—those solitary 10 minutes each night before you fall asleep. For it is in those 10 minutes that you find yourself utterly and completely alone with your life; what you said, and what you meant to say; what you did, and what you didn’t do. Despite the ups and downs of the year, I’ve never once begrudged those 10 minutes, for I have nothing but pride in my heart as I think about you and SHM, an organization and a community that is, and will continue to be, one that cares about the right things in life.

So this is me signing off. I look forward to serving HM in whatever way I can as the years transpire.

For now, I look forward to president-elect Joseph Ming Wah Li, MD, SFHM, FACP, continuing this journey. And so should you. I am confident that even better days are soon to come under his leadership. TH

Dr. Wiese is president of SHM.

It’s been one of those days. It all started at 4:30 this morning, when my 3-year-old son crawled into our bed, naked except for the diarrhea dripping down his leg. Turns out, this was his way—quite effective, I might add—of telling my wife and me that he had had an “accident.” After an hour of carrying soiled sheets to the washer, child-bathing, and Weimaraner coat-scrubbing, we relaxed to the sound of our 1-year-old daughter’s blood-curdling screams.

Upon examination, we found that the night had mysteriously transformed our precious little button-nosed bundle of joy into a tangle-haired, snot-nosed bundle of melancholy. Where her face used to be, there now hung something approximating the mask from that Scream movie. Additionally, her throat was raw, olive-sized lymph nodes populated her neck, and her nose had taken to perpetual booger-manufacturing. A rapid strep swab would later reveal the culprit, but at the moment, our differential tilted toward demonic possession.

That Dripping Feeling

Moments later, my wife and I picked 6:15 a.m. as the time to discover that we both had 7 a.m. meetings and no time to drop the kids off at daycare, especially when factoring in the 10-minute “discussion” we had about who was going to drop the kids off at daycare. All of this preceded my 7:10 a.m. arrival time for the 7 o’clock meeting with a hospital executive team to discuss our HM group funding for the next year—an encounter that left me feeling as my son must have just prior to crawling into bed with us that morning.

Now 8 a.m., I had to meet with a surgeon eager to unveil his “great idea” for our hospitalists to admit all of his patients. “It solves our problem of no interns, and allows you to play a meaningful role in the hospital!” he exclaimed.

“The meaningful role of intern?” I replied. Again, I had that dripping feeling.

It was 8:30 a.m. and I was ready to round on my patients. The first patient, a lovely woman, was stricken with un-insure-ia and a deep-seated belief that the inequitable health system that rendered her unable to get her surgery was clearly the result of some moral failing on my part. Next up was a spectacularly intoxicated male who welcomed my caring touch by belching a bit of breakfast burrito onto my cheek. Then it was a floridly bipolar patient whose apparent life mission was to drop her pants to show me her new mesh thong.

Burnout, Respect, Satisfaction

And so it continued until 1 p.m., when I had a meeting with a resident mentee of mine. It turns out that he wanted to tell me that despite his desire to be a hospitalist since his fourth year of medical school, he instead was going to apply to a rheumatology fellowship. After talking to several practicing hospitalists, he’d decided it just wasn’t for him—discussions he summarized as too much burnout, too little respect, and not enough satisfaction. Again, that dripping feeling.

Stuffing my face with a vending-machine carb-load that doubled as both breakfast and lunch, I sat down for a few minutes of e-mail. First up, a journal rejection of a research paper we’d recently submitted. Oh, the fulfillment of academics. Next were two e-mails that enzymatically trebled my “to do” list for the day. Sandwiched between those e-mails and one from a friend reminding me not to be late for a dinner that night that I was clearly going to be late for was an e-mail from a nice-appearing Nigerian man wanting to give me millions of dollars; at last, my day was turning around.

Alas, this was not the case. Checking my voicemail, I found out that my uncle was in the hospital, my dog’s lab tests were abnormal, my mom was angry about something, and I had missed a dentist appointment that morning. Finally, our group assistant came with a message that our prized hospitalist recruit had accepted a job at another institution. Drip … drip … drip…

Self-Reflection

Now 2:30 p.m., I took stock of my day and reflected on what my resident mentee had said about hospitalists. Trying to balance the rigors of patient care, academic requirements, life, friends, family, and being a boss, I was most definitely feeling a bit downtrodden, unsated, and crispy around the edges. What, exactly, did I like about this job? Was this what I wanted professionally? Would I ever find balance? Perhaps, too, a rheumatology application could salve my problems.

It was at that point that the notice for the SHM annual meeting appeared, oracle-like, on my desk. Picking it up, I realized this, not a two-year sojourn through the world of creaky joints, was the tonic to my problems.

Meeting Hierarchy

Every year since I began going to the SHM annual meeting in 2003, the meeting has helped me rejuvenate and grow. Much like Maslow’s Hierarchy of Needs, which posits that humans develop in stages that build on each other, I’ve found stepwise growth in the annual meeting.

Before my first meeting, I had been wandering nomadically through a hospitalist job for three years, wondering what, exactly, I was doing. I was the only hospitalist in my group, had few days off, with no support system around me. I had just agreed to take a job at another institution to build a new 10-person hospitalist group and had no idea how to do this. In Maslow terms, I was trying to satisfy my “physiologic needs” to survive. I needed to find, metaphorically, “food, water, clothes, and shelter.”

I found them at the annual meeting. The practice-management pre-course taught me how to build a hospitalist group, the mentorship breakfast introduced me to a veteran I still turn to, and the educational offerings helped improve my patient-care skills. I had conquered the base of Maslow’s pyramid.

The next year, I became involved in an SHM committee, and our gathering at the annual meeting helped set the course for our group’s future endeavors. I also met up with many friends I hadn’t seen since medical school and even recruited a person to my new group. In Maslow-speak, the meeting was helping me achieve my “safety needs” by providing control, well-being, and predictability.

By the third year, I was beginning to look forward to meeting up with national colleagues I had met at prior annual meetings, fulfilling Maslow’s third-stage need of “belonging.” During the ensuing years, I presented research projects, gave talks, and helped develop and lead forums and summits, thus quenching Maslow’s “self-esteem” need.

I wonder, as I leave my office to go back to see my afternoon complement of new patients, what my ninth annual meeting will bring. I’m not sure if I’ll ever achieve Maslow’s final phase of “self-actualization,” mostly because I’m not entirely sure what that means. However, I do know this: This job can be tough. We all feel it regardless of our age, gender, or practice setting. It is easy to get knocked out of balance, to get beaten down, to lose our focus. It is at those times that we all need a mariner to right the course. To remind us why we do this, to allow us to recharge, to facilitate our growth, to fulfill our needs.

For me, that mariner is the annual meeting. I look forward to seeing you all in Dallas next month. TH

Dr. Glasheen is associate professor of medicine at the University of Colorado at Denver, where he serves as director of the Hospital Medicine Program and the Hospitalist Training Program, and as associate program director of the Internal Medicine Residency Program.

It’s been one of those days. It all started at 4:30 this morning, when my 3-year-old son crawled into our bed, naked except for the diarrhea dripping down his leg. Turns out, this was his way—quite effective, I might add—of telling my wife and me that he had had an “accident.” After an hour of carrying soiled sheets to the washer, child-bathing, and Weimaraner coat-scrubbing, we relaxed to the sound of our 1-year-old daughter’s blood-curdling screams.

Upon examination, we found that the night had mysteriously transformed our precious little button-nosed bundle of joy into a tangle-haired, snot-nosed bundle of melancholy. Where her face used to be, there now hung something approximating the mask from that Scream movie. Additionally, her throat was raw, olive-sized lymph nodes populated her neck, and her nose had taken to perpetual booger-manufacturing. A rapid strep swab would later reveal the culprit, but at the moment, our differential tilted toward demonic possession.

That Dripping Feeling

Moments later, my wife and I picked 6:15 a.m. as the time to discover that we both had 7 a.m. meetings and no time to drop the kids off at daycare, especially when factoring in the 10-minute “discussion” we had about who was going to drop the kids off at daycare. All of this preceded my 7:10 a.m. arrival time for the 7 o’clock meeting with a hospital executive team to discuss our HM group funding for the next year—an encounter that left me feeling as my son must have just prior to crawling into bed with us that morning.

Now 8 a.m., I had to meet with a surgeon eager to unveil his “great idea” for our hospitalists to admit all of his patients. “It solves our problem of no interns, and allows you to play a meaningful role in the hospital!” he exclaimed.

“The meaningful role of intern?” I replied. Again, I had that dripping feeling.

It was 8:30 a.m. and I was ready to round on my patients. The first patient, a lovely woman, was stricken with un-insure-ia and a deep-seated belief that the inequitable health system that rendered her unable to get her surgery was clearly the result of some moral failing on my part. Next up was a spectacularly intoxicated male who welcomed my caring touch by belching a bit of breakfast burrito onto my cheek. Then it was a floridly bipolar patient whose apparent life mission was to drop her pants to show me her new mesh thong.

Burnout, Respect, Satisfaction

And so it continued until 1 p.m., when I had a meeting with a resident mentee of mine. It turns out that he wanted to tell me that despite his desire to be a hospitalist since his fourth year of medical school, he instead was going to apply to a rheumatology fellowship. After talking to several practicing hospitalists, he’d decided it just wasn’t for him—discussions he summarized as too much burnout, too little respect, and not enough satisfaction. Again, that dripping feeling.

Stuffing my face with a vending-machine carb-load that doubled as both breakfast and lunch, I sat down for a few minutes of e-mail. First up, a journal rejection of a research paper we’d recently submitted. Oh, the fulfillment of academics. Next were two e-mails that enzymatically trebled my “to do” list for the day. Sandwiched between those e-mails and one from a friend reminding me not to be late for a dinner that night that I was clearly going to be late for was an e-mail from a nice-appearing Nigerian man wanting to give me millions of dollars; at last, my day was turning around.

Alas, this was not the case. Checking my voicemail, I found out that my uncle was in the hospital, my dog’s lab tests were abnormal, my mom was angry about something, and I had missed a dentist appointment that morning. Finally, our group assistant came with a message that our prized hospitalist recruit had accepted a job at another institution. Drip … drip … drip…

Self-Reflection

Now 2:30 p.m., I took stock of my day and reflected on what my resident mentee had said about hospitalists. Trying to balance the rigors of patient care, academic requirements, life, friends, family, and being a boss, I was most definitely feeling a bit downtrodden, unsated, and crispy around the edges. What, exactly, did I like about this job? Was this what I wanted professionally? Would I ever find balance? Perhaps, too, a rheumatology application could salve my problems.

It was at that point that the notice for the SHM annual meeting appeared, oracle-like, on my desk. Picking it up, I realized this, not a two-year sojourn through the world of creaky joints, was the tonic to my problems.

Meeting Hierarchy

Every year since I began going to the SHM annual meeting in 2003, the meeting has helped me rejuvenate and grow. Much like Maslow’s Hierarchy of Needs, which posits that humans develop in stages that build on each other, I’ve found stepwise growth in the annual meeting.

Before my first meeting, I had been wandering nomadically through a hospitalist job for three years, wondering what, exactly, I was doing. I was the only hospitalist in my group, had few days off, with no support system around me. I had just agreed to take a job at another institution to build a new 10-person hospitalist group and had no idea how to do this. In Maslow terms, I was trying to satisfy my “physiologic needs” to survive. I needed to find, metaphorically, “food, water, clothes, and shelter.”

I found them at the annual meeting. The practice-management pre-course taught me how to build a hospitalist group, the mentorship breakfast introduced me to a veteran I still turn to, and the educational offerings helped improve my patient-care skills. I had conquered the base of Maslow’s pyramid.

The next year, I became involved in an SHM committee, and our gathering at the annual meeting helped set the course for our group’s future endeavors. I also met up with many friends I hadn’t seen since medical school and even recruited a person to my new group. In Maslow-speak, the meeting was helping me achieve my “safety needs” by providing control, well-being, and predictability.

By the third year, I was beginning to look forward to meeting up with national colleagues I had met at prior annual meetings, fulfilling Maslow’s third-stage need of “belonging.” During the ensuing years, I presented research projects, gave talks, and helped develop and lead forums and summits, thus quenching Maslow’s “self-esteem” need.

I wonder, as I leave my office to go back to see my afternoon complement of new patients, what my ninth annual meeting will bring. I’m not sure if I’ll ever achieve Maslow’s final phase of “self-actualization,” mostly because I’m not entirely sure what that means. However, I do know this: This job can be tough. We all feel it regardless of our age, gender, or practice setting. It is easy to get knocked out of balance, to get beaten down, to lose our focus. It is at those times that we all need a mariner to right the course. To remind us why we do this, to allow us to recharge, to facilitate our growth, to fulfill our needs.

For me, that mariner is the annual meeting. I look forward to seeing you all in Dallas next month. TH

Dr. Glasheen is associate professor of medicine at the University of Colorado at Denver, where he serves as director of the Hospital Medicine Program and the Hospitalist Training Program, and as associate program director of the Internal Medicine Residency Program.

It’s been one of those days. It all started at 4:30 this morning, when my 3-year-old son crawled into our bed, naked except for the diarrhea dripping down his leg. Turns out, this was his way—quite effective, I might add—of telling my wife and me that he had had an “accident.” After an hour of carrying soiled sheets to the washer, child-bathing, and Weimaraner coat-scrubbing, we relaxed to the sound of our 1-year-old daughter’s blood-curdling screams.

Upon examination, we found that the night had mysteriously transformed our precious little button-nosed bundle of joy into a tangle-haired, snot-nosed bundle of melancholy. Where her face used to be, there now hung something approximating the mask from that Scream movie. Additionally, her throat was raw, olive-sized lymph nodes populated her neck, and her nose had taken to perpetual booger-manufacturing. A rapid strep swab would later reveal the culprit, but at the moment, our differential tilted toward demonic possession.

That Dripping Feeling

Moments later, my wife and I picked 6:15 a.m. as the time to discover that we both had 7 a.m. meetings and no time to drop the kids off at daycare, especially when factoring in the 10-minute “discussion” we had about who was going to drop the kids off at daycare. All of this preceded my 7:10 a.m. arrival time for the 7 o’clock meeting with a hospital executive team to discuss our HM group funding for the next year—an encounter that left me feeling as my son must have just prior to crawling into bed with us that morning.

Now 8 a.m., I had to meet with a surgeon eager to unveil his “great idea” for our hospitalists to admit all of his patients. “It solves our problem of no interns, and allows you to play a meaningful role in the hospital!” he exclaimed.

“The meaningful role of intern?” I replied. Again, I had that dripping feeling.

It was 8:30 a.m. and I was ready to round on my patients. The first patient, a lovely woman, was stricken with un-insure-ia and a deep-seated belief that the inequitable health system that rendered her unable to get her surgery was clearly the result of some moral failing on my part. Next up was a spectacularly intoxicated male who welcomed my caring touch by belching a bit of breakfast burrito onto my cheek. Then it was a floridly bipolar patient whose apparent life mission was to drop her pants to show me her new mesh thong.

Burnout, Respect, Satisfaction

And so it continued until 1 p.m., when I had a meeting with a resident mentee of mine. It turns out that he wanted to tell me that despite his desire to be a hospitalist since his fourth year of medical school, he instead was going to apply to a rheumatology fellowship. After talking to several practicing hospitalists, he’d decided it just wasn’t for him—discussions he summarized as too much burnout, too little respect, and not enough satisfaction. Again, that dripping feeling.

Stuffing my face with a vending-machine carb-load that doubled as both breakfast and lunch, I sat down for a few minutes of e-mail. First up, a journal rejection of a research paper we’d recently submitted. Oh, the fulfillment of academics. Next were two e-mails that enzymatically trebled my “to do” list for the day. Sandwiched between those e-mails and one from a friend reminding me not to be late for a dinner that night that I was clearly going to be late for was an e-mail from a nice-appearing Nigerian man wanting to give me millions of dollars; at last, my day was turning around.

Alas, this was not the case. Checking my voicemail, I found out that my uncle was in the hospital, my dog’s lab tests were abnormal, my mom was angry about something, and I had missed a dentist appointment that morning. Finally, our group assistant came with a message that our prized hospitalist recruit had accepted a job at another institution. Drip … drip … drip…

Self-Reflection

Now 2:30 p.m., I took stock of my day and reflected on what my resident mentee had said about hospitalists. Trying to balance the rigors of patient care, academic requirements, life, friends, family, and being a boss, I was most definitely feeling a bit downtrodden, unsated, and crispy around the edges. What, exactly, did I like about this job? Was this what I wanted professionally? Would I ever find balance? Perhaps, too, a rheumatology application could salve my problems.

It was at that point that the notice for the SHM annual meeting appeared, oracle-like, on my desk. Picking it up, I realized this, not a two-year sojourn through the world of creaky joints, was the tonic to my problems.

Meeting Hierarchy

Every year since I began going to the SHM annual meeting in 2003, the meeting has helped me rejuvenate and grow. Much like Maslow’s Hierarchy of Needs, which posits that humans develop in stages that build on each other, I’ve found stepwise growth in the annual meeting.

Before my first meeting, I had been wandering nomadically through a hospitalist job for three years, wondering what, exactly, I was doing. I was the only hospitalist in my group, had few days off, with no support system around me. I had just agreed to take a job at another institution to build a new 10-person hospitalist group and had no idea how to do this. In Maslow terms, I was trying to satisfy my “physiologic needs” to survive. I needed to find, metaphorically, “food, water, clothes, and shelter.”

I found them at the annual meeting. The practice-management pre-course taught me how to build a hospitalist group, the mentorship breakfast introduced me to a veteran I still turn to, and the educational offerings helped improve my patient-care skills. I had conquered the base of Maslow’s pyramid.

The next year, I became involved in an SHM committee, and our gathering at the annual meeting helped set the course for our group’s future endeavors. I also met up with many friends I hadn’t seen since medical school and even recruited a person to my new group. In Maslow-speak, the meeting was helping me achieve my “safety needs” by providing control, well-being, and predictability.

By the third year, I was beginning to look forward to meeting up with national colleagues I had met at prior annual meetings, fulfilling Maslow’s third-stage need of “belonging.” During the ensuing years, I presented research projects, gave talks, and helped develop and lead forums and summits, thus quenching Maslow’s “self-esteem” need.

I wonder, as I leave my office to go back to see my afternoon complement of new patients, what my ninth annual meeting will bring. I’m not sure if I’ll ever achieve Maslow’s final phase of “self-actualization,” mostly because I’m not entirely sure what that means. However, I do know this: This job can be tough. We all feel it regardless of our age, gender, or practice setting. It is easy to get knocked out of balance, to get beaten down, to lose our focus. It is at those times that we all need a mariner to right the course. To remind us why we do this, to allow us to recharge, to facilitate our growth, to fulfill our needs.

For me, that mariner is the annual meeting. I look forward to seeing you all in Dallas next month. TH

Dr. Glasheen is associate professor of medicine at the University of Colorado at Denver, where he serves as director of the Hospital Medicine Program and the Hospitalist Training Program, and as associate program director of the Internal Medicine Residency Program.

Last month, I wrote about the attributes of hospitalist practices that I associate with success. This month, I’ll do the opposite. That is, I’ll write about strategies your practice could, or even should, do without. Of course, all of these things are open to debate, and some thoughtful people might (and in my experience, probably will) arrive at different conclusions.

So I offer my list as food for thought, and if your practice relies on some of these strategies, you shouldn’t feel threatened by my opinion. But you might want to think about whether they’ve been made part of your practice by design, or if things just evolved this way without careful consideration of alternatives. I’ve listed them in no particular order.

Fixed-duration day shifts. My sense is that the majority of practices have a day shift with a predetermined start and end. That is, the hospitalist is expected to arrive and depart at the same time each day.

This seems to make a lot of sense, but it ignores the dramatic variations in workload a practice will have. For example, a practice that is appropriately staffed with four daytime hospitalists, and schedules each of them to work a 12-hour shift, provides 48 hours of daytime hospitalist manpower each day. But that will turn out to be precisely the right level of staffing only a few days a year. On all other days, daytime staffing will be optimal with a different number of hours. So it would make sense for the doctors to work more or less on those days.

Telling doctors that their shift always starts at the same time has significant lifestyle advantages. But it can inhibit the doctors who would be happy to start earlier to address more discharges early in the day and potentially go home earlier. So, just like most other doctors at your hospital have, why not let the doctors have significant latitude in when they start and stop working each day? In most cases, it might be necessary to have a time by which every doctor must be available to respond to pages (and one who must be on-site before the night doctor leaves), but they should feel free to actually arrive and start working when they choose. Most will make good choices and will likely feel a little more empowered and happy with their work.

And, at the end of the day, it might be reasonable to allow some of the day-shift doctors to leave when their work is done, and allow the others to stay to handle admissions until the night shift takes over. Those who leave early might still be required to respond to pages until a specified time.

Shifts that don’t involve rounding on “continuity” patients, such as night and evening (“swing”) shifts, usually should be arranged with predetermined start. I wrote in more detail on this topic in January 2007 and October 2010.

Contractual vacation provisions. Hospitalists should have significant amounts of time off. We work a lot of evenings, nights, and weekends, and we must have liberal amounts of time away from work. But for many practices, there is no advantage in classifying this time as vacation (or CME, etc.) time. In most cases, it makes the most sense to simply specify how much work (e.g. number of shifts) a doctor is to do each year and not specify a number of days or hours of vacation time. For more detail, read “The Vacation Conundrum” from March 2007.

If your practice has a vacation system that works well, then stick with it. But if you or your administrators are going nuts trying to categorize nonworking days between vacation and days the doctor simply wasn’t scheduled, then it might be best to stop trying. Just settle on the number of shifts (or some other metric) that a doctor is to work each year.

Tenure-based salary increases. It makes a lot of sense to pay doctors in most specialties an increasing salary based on his or her tenure with the practice. As they build a patient population and a referral stream, they generate more revenue and should benefit accordingly. But a new hospitalist who joins an existing group almost never has to build the referrals. In most cases, the group hired the doctor because the referrals are already coming and the practice needs more help, or the new doctor is replacing a departing one. So paying a new hospitalist a lower salary that increases automatically every few years isn’t really a raise earned by the doctor’s improved financial performance. Usually it’s just a system of withholding money that could be available for compensation for the doctor’s first few years in the practice. This lower starting salary might adversely impact recruiting. For more, see “Compensation Conundrum” from December 2009.

Poor roles for nonphysician providers (NPPs). I’ve worked with a lot of practices that have NPs and PAs (and, in some cases, RNs) who are doing what amounts to clerical work. They’re faxing discharge summaries, making calls to schedule patient appointments, dividing up the overnight admissions for the day rounders, etc.

Don’t make this mistake. Hire a secretary for that sort of work. And be sure that the roles occupied by trained clinicians (PAs, NPs, RNs, etc.) are professionally satisfying and will position them to make an effective contribution to the practice.

For more on this topic, see “The 411 on NPPs” from September 2008 and “Role Refinement” from September 2009; the latter features the perspective of Ryan Genzink, a thoughtful PA-C from Michigan.

Blinded performance reporting. First, make sure your practice provides regular, meaningful reports on each doctor’s performance and the group as a whole. This usually takes the form of a dashboard or report card. In my experience, too few practices do this. Make sure your group isn’t in that category.

Groups that do provide performance data often allow each doctor to see only his or her data. If data about other individuals in the group are provided, the names have often been removed. With exception of certain human resources issues (e.g. counseling a doctor to prevent termination), I think all performance data in the group should be shared by name with the whole group. In most practices, everyone should know by name which doctors are the high and low producers, each doctor’s compensation, and CPT coding practices (e.g. the portion of discharges coded at the high level).

When clinical performance can be attributed to individual providers, report those metrics openly, too. This usually creates greater cohesion within the group and helps foster a mentality of practice ownership. TH

Dr. Nelson has been a practicing hospitalist since 1988 and is co-founder and past president of SHM. He is a principal in Nelson Flores Hospital Medicine Consultants a national hospitalist practice management consulting firm (www.nelsonflores.com). He is course co-director and faculty for SHM’s “Best Practices in Managing a Hospital Medicine Program.” This column represents his views and is not intended to reflect an official position of SHM.

Last month, I wrote about the attributes of hospitalist practices that I associate with success. This month, I’ll do the opposite. That is, I’ll write about strategies your practice could, or even should, do without. Of course, all of these things are open to debate, and some thoughtful people might (and in my experience, probably will) arrive at different conclusions.

So I offer my list as food for thought, and if your practice relies on some of these strategies, you shouldn’t feel threatened by my opinion. But you might want to think about whether they’ve been made part of your practice by design, or if things just evolved this way without careful consideration of alternatives. I’ve listed them in no particular order.

Fixed-duration day shifts. My sense is that the majority of practices have a day shift with a predetermined start and end. That is, the hospitalist is expected to arrive and depart at the same time each day.

This seems to make a lot of sense, but it ignores the dramatic variations in workload a practice will have. For example, a practice that is appropriately staffed with four daytime hospitalists, and schedules each of them to work a 12-hour shift, provides 48 hours of daytime hospitalist manpower each day. But that will turn out to be precisely the right level of staffing only a few days a year. On all other days, daytime staffing will be optimal with a different number of hours. So it would make sense for the doctors to work more or less on those days.

Telling doctors that their shift always starts at the same time has significant lifestyle advantages. But it can inhibit the doctors who would be happy to start earlier to address more discharges early in the day and potentially go home earlier. So, just like most other doctors at your hospital have, why not let the doctors have significant latitude in when they start and stop working each day? In most cases, it might be necessary to have a time by which every doctor must be available to respond to pages (and one who must be on-site before the night doctor leaves), but they should feel free to actually arrive and start working when they choose. Most will make good choices and will likely feel a little more empowered and happy with their work.

And, at the end of the day, it might be reasonable to allow some of the day-shift doctors to leave when their work is done, and allow the others to stay to handle admissions until the night shift takes over. Those who leave early might still be required to respond to pages until a specified time.

Shifts that don’t involve rounding on “continuity” patients, such as night and evening (“swing”) shifts, usually should be arranged with predetermined start. I wrote in more detail on this topic in January 2007 and October 2010.

Contractual vacation provisions. Hospitalists should have significant amounts of time off. We work a lot of evenings, nights, and weekends, and we must have liberal amounts of time away from work. But for many practices, there is no advantage in classifying this time as vacation (or CME, etc.) time. In most cases, it makes the most sense to simply specify how much work (e.g. number of shifts) a doctor is to do each year and not specify a number of days or hours of vacation time. For more detail, read “The Vacation Conundrum” from March 2007.

If your practice has a vacation system that works well, then stick with it. But if you or your administrators are going nuts trying to categorize nonworking days between vacation and days the doctor simply wasn’t scheduled, then it might be best to stop trying. Just settle on the number of shifts (or some other metric) that a doctor is to work each year.

Tenure-based salary increases. It makes a lot of sense to pay doctors in most specialties an increasing salary based on his or her tenure with the practice. As they build a patient population and a referral stream, they generate more revenue and should benefit accordingly. But a new hospitalist who joins an existing group almost never has to build the referrals. In most cases, the group hired the doctor because the referrals are already coming and the practice needs more help, or the new doctor is replacing a departing one. So paying a new hospitalist a lower salary that increases automatically every few years isn’t really a raise earned by the doctor’s improved financial performance. Usually it’s just a system of withholding money that could be available for compensation for the doctor’s first few years in the practice. This lower starting salary might adversely impact recruiting. For more, see “Compensation Conundrum” from December 2009.

Poor roles for nonphysician providers (NPPs). I’ve worked with a lot of practices that have NPs and PAs (and, in some cases, RNs) who are doing what amounts to clerical work. They’re faxing discharge summaries, making calls to schedule patient appointments, dividing up the overnight admissions for the day rounders, etc.

Don’t make this mistake. Hire a secretary for that sort of work. And be sure that the roles occupied by trained clinicians (PAs, NPs, RNs, etc.) are professionally satisfying and will position them to make an effective contribution to the practice.

For more on this topic, see “The 411 on NPPs” from September 2008 and “Role Refinement” from September 2009; the latter features the perspective of Ryan Genzink, a thoughtful PA-C from Michigan.

Blinded performance reporting. First, make sure your practice provides regular, meaningful reports on each doctor’s performance and the group as a whole. This usually takes the form of a dashboard or report card. In my experience, too few practices do this. Make sure your group isn’t in that category.

Groups that do provide performance data often allow each doctor to see only his or her data. If data about other individuals in the group are provided, the names have often been removed. With exception of certain human resources issues (e.g. counseling a doctor to prevent termination), I think all performance data in the group should be shared by name with the whole group. In most practices, everyone should know by name which doctors are the high and low producers, each doctor’s compensation, and CPT coding practices (e.g. the portion of discharges coded at the high level).

When clinical performance can be attributed to individual providers, report those metrics openly, too. This usually creates greater cohesion within the group and helps foster a mentality of practice ownership. TH

Dr. Nelson has been a practicing hospitalist since 1988 and is co-founder and past president of SHM. He is a principal in Nelson Flores Hospital Medicine Consultants a national hospitalist practice management consulting firm (www.nelsonflores.com). He is course co-director and faculty for SHM’s “Best Practices in Managing a Hospital Medicine Program.” This column represents his views and is not intended to reflect an official position of SHM.

Last month, I wrote about the attributes of hospitalist practices that I associate with success. This month, I’ll do the opposite. That is, I’ll write about strategies your practice could, or even should, do without. Of course, all of these things are open to debate, and some thoughtful people might (and in my experience, probably will) arrive at different conclusions.

So I offer my list as food for thought, and if your practice relies on some of these strategies, you shouldn’t feel threatened by my opinion. But you might want to think about whether they’ve been made part of your practice by design, or if things just evolved this way without careful consideration of alternatives. I’ve listed them in no particular order.

Fixed-duration day shifts. My sense is that the majority of practices have a day shift with a predetermined start and end. That is, the hospitalist is expected to arrive and depart at the same time each day.

This seems to make a lot of sense, but it ignores the dramatic variations in workload a practice will have. For example, a practice that is appropriately staffed with four daytime hospitalists, and schedules each of them to work a 12-hour shift, provides 48 hours of daytime hospitalist manpower each day. But that will turn out to be precisely the right level of staffing only a few days a year. On all other days, daytime staffing will be optimal with a different number of hours. So it would make sense for the doctors to work more or less on those days.

Telling doctors that their shift always starts at the same time has significant lifestyle advantages. But it can inhibit the doctors who would be happy to start earlier to address more discharges early in the day and potentially go home earlier. So, just like most other doctors at your hospital have, why not let the doctors have significant latitude in when they start and stop working each day? In most cases, it might be necessary to have a time by which every doctor must be available to respond to pages (and one who must be on-site before the night doctor leaves), but they should feel free to actually arrive and start working when they choose. Most will make good choices and will likely feel a little more empowered and happy with their work.

And, at the end of the day, it might be reasonable to allow some of the day-shift doctors to leave when their work is done, and allow the others to stay to handle admissions until the night shift takes over. Those who leave early might still be required to respond to pages until a specified time.

Shifts that don’t involve rounding on “continuity” patients, such as night and evening (“swing”) shifts, usually should be arranged with predetermined start. I wrote in more detail on this topic in January 2007 and October 2010.

Contractual vacation provisions. Hospitalists should have significant amounts of time off. We work a lot of evenings, nights, and weekends, and we must have liberal amounts of time away from work. But for many practices, there is no advantage in classifying this time as vacation (or CME, etc.) time. In most cases, it makes the most sense to simply specify how much work (e.g. number of shifts) a doctor is to do each year and not specify a number of days or hours of vacation time. For more detail, read “The Vacation Conundrum” from March 2007.

If your practice has a vacation system that works well, then stick with it. But if you or your administrators are going nuts trying to categorize nonworking days between vacation and days the doctor simply wasn’t scheduled, then it might be best to stop trying. Just settle on the number of shifts (or some other metric) that a doctor is to work each year.

Tenure-based salary increases. It makes a lot of sense to pay doctors in most specialties an increasing salary based on his or her tenure with the practice. As they build a patient population and a referral stream, they generate more revenue and should benefit accordingly. But a new hospitalist who joins an existing group almost never has to build the referrals. In most cases, the group hired the doctor because the referrals are already coming and the practice needs more help, or the new doctor is replacing a departing one. So paying a new hospitalist a lower salary that increases automatically every few years isn’t really a raise earned by the doctor’s improved financial performance. Usually it’s just a system of withholding money that could be available for compensation for the doctor’s first few years in the practice. This lower starting salary might adversely impact recruiting. For more, see “Compensation Conundrum” from December 2009.

Poor roles for nonphysician providers (NPPs). I’ve worked with a lot of practices that have NPs and PAs (and, in some cases, RNs) who are doing what amounts to clerical work. They’re faxing discharge summaries, making calls to schedule patient appointments, dividing up the overnight admissions for the day rounders, etc.

Don’t make this mistake. Hire a secretary for that sort of work. And be sure that the roles occupied by trained clinicians (PAs, NPs, RNs, etc.) are professionally satisfying and will position them to make an effective contribution to the practice.

For more on this topic, see “The 411 on NPPs” from September 2008 and “Role Refinement” from September 2009; the latter features the perspective of Ryan Genzink, a thoughtful PA-C from Michigan.

Blinded performance reporting. First, make sure your practice provides regular, meaningful reports on each doctor’s performance and the group as a whole. This usually takes the form of a dashboard or report card. In my experience, too few practices do this. Make sure your group isn’t in that category.

Groups that do provide performance data often allow each doctor to see only his or her data. If data about other individuals in the group are provided, the names have often been removed. With exception of certain human resources issues (e.g. counseling a doctor to prevent termination), I think all performance data in the group should be shared by name with the whole group. In most practices, everyone should know by name which doctors are the high and low producers, each doctor’s compensation, and CPT coding practices (e.g. the portion of discharges coded at the high level).

When clinical performance can be attributed to individual providers, report those metrics openly, too. This usually creates greater cohesion within the group and helps foster a mentality of practice ownership. TH

Dr. Nelson has been a practicing hospitalist since 1988 and is co-founder and past president of SHM. He is a principal in Nelson Flores Hospital Medicine Consultants a national hospitalist practice management consulting firm (www.nelsonflores.com). He is course co-director and faculty for SHM’s “Best Practices in Managing a Hospital Medicine Program.” This column represents his views and is not intended to reflect an official position of SHM.

I heard from my hospital’s pharmacist director that soon I will not be able to prescribe Epogen unless I get some additional certification. Is this true? Can you explain what is going on?

Ritesh Magge, MD,

Detroit

Dr. Hospitalist responds: Your pharmacist is referring to the new FDA-mandated requirements, which went into effect in February, that will affect who can prescribe erythropoesis-stimulating agents (ESAs), including epoetin (Epogen, Procrit) and darbepoetin (Aranesp). This is part of a broader FDA strategy to improve patient safety as it relates to medications.

According to the FDA website, “The Food and Drug Administration Amendments Act of 2007 gave FDA the authority to require a Risk Evaluation and Mitigation Strategy (REMS) from manufacturers to ensure that the benefits of a drug or biological product outweigh its risks.”

The ESAs are not the only class of drug that will require a REM. More than 150 drugs already have varying requirements. For a complete list of these medications, please visit the FDA website (www.fda.gov) and search “drug safety.” The FDA plans to create additional REMs for additional drugs and biologic agents.

If you are a provider who will prescribe a medication with a REM, you will be obligated to adhere to the FDA REM associated with that medication. The drug manufacturers are working with the FDA to set up REMs for their drugs. For example, if you plan to prescribe an ESA for a patient with cancer, you will first need to enroll in the ESA APPRISE (Assisting Providers and Cancer Patients with Risk Information for the Safe Use of ESAs) Oncology Program, which was established by ESA drug manufacturers. Once enrolled in the program, you will be assigned a unique ESA APPRISE ID number. ESA prescribers will be required to provide and review the ESA Medication Guide and counsel each patient on the risks and benefits of ESAs before each course of therapy. This counseling must be done no more than every 30 days after the first dose. (At the same time, prescribing ESAs to non-cancer patients basically only entails patient education.)

Providers also will be required to fill out the ESA APPRISE Oncology Program Patient and Healthcare Professional Acknowledgment form, which documents that the risk-benefit discussion occurred. This form is in triplicate and includes both the patient and the provider signature. One copy is given to the patient, another copy placed in the chart, and the third copy is stored for potential future audit.

For more information about the ESA APPRISE Oncology Program, please visit www.esaapprise.com/ESAAppriseUI/ESAAppriseUI/default.jsp. TH

I heard from my hospital’s pharmacist director that soon I will not be able to prescribe Epogen unless I get some additional certification. Is this true? Can you explain what is going on?

Ritesh Magge, MD,

Detroit

Dr. Hospitalist responds: Your pharmacist is referring to the new FDA-mandated requirements, which went into effect in February, that will affect who can prescribe erythropoesis-stimulating agents (ESAs), including epoetin (Epogen, Procrit) and darbepoetin (Aranesp). This is part of a broader FDA strategy to improve patient safety as it relates to medications.

According to the FDA website, “The Food and Drug Administration Amendments Act of 2007 gave FDA the authority to require a Risk Evaluation and Mitigation Strategy (REMS) from manufacturers to ensure that the benefits of a drug or biological product outweigh its risks.”

The ESAs are not the only class of drug that will require a REM. More than 150 drugs already have varying requirements. For a complete list of these medications, please visit the FDA website (www.fda.gov) and search “drug safety.” The FDA plans to create additional REMs for additional drugs and biologic agents.

If you are a provider who will prescribe a medication with a REM, you will be obligated to adhere to the FDA REM associated with that medication. The drug manufacturers are working with the FDA to set up REMs for their drugs. For example, if you plan to prescribe an ESA for a patient with cancer, you will first need to enroll in the ESA APPRISE (Assisting Providers and Cancer Patients with Risk Information for the Safe Use of ESAs) Oncology Program, which was established by ESA drug manufacturers. Once enrolled in the program, you will be assigned a unique ESA APPRISE ID number. ESA prescribers will be required to provide and review the ESA Medication Guide and counsel each patient on the risks and benefits of ESAs before each course of therapy. This counseling must be done no more than every 30 days after the first dose. (At the same time, prescribing ESAs to non-cancer patients basically only entails patient education.)

Providers also will be required to fill out the ESA APPRISE Oncology Program Patient and Healthcare Professional Acknowledgment form, which documents that the risk-benefit discussion occurred. This form is in triplicate and includes both the patient and the provider signature. One copy is given to the patient, another copy placed in the chart, and the third copy is stored for potential future audit.

For more information about the ESA APPRISE Oncology Program, please visit www.esaapprise.com/ESAAppriseUI/ESAAppriseUI/default.jsp. TH

I heard from my hospital’s pharmacist director that soon I will not be able to prescribe Epogen unless I get some additional certification. Is this true? Can you explain what is going on?

Ritesh Magge, MD,

Detroit

Dr. Hospitalist responds: Your pharmacist is referring to the new FDA-mandated requirements, which went into effect in February, that will affect who can prescribe erythropoesis-stimulating agents (ESAs), including epoetin (Epogen, Procrit) and darbepoetin (Aranesp). This is part of a broader FDA strategy to improve patient safety as it relates to medications.

According to the FDA website, “The Food and Drug Administration Amendments Act of 2007 gave FDA the authority to require a Risk Evaluation and Mitigation Strategy (REMS) from manufacturers to ensure that the benefits of a drug or biological product outweigh its risks.”

The ESAs are not the only class of drug that will require a REM. More than 150 drugs already have varying requirements. For a complete list of these medications, please visit the FDA website (www.fda.gov) and search “drug safety.” The FDA plans to create additional REMs for additional drugs and biologic agents.

If you are a provider who will prescribe a medication with a REM, you will be obligated to adhere to the FDA REM associated with that medication. The drug manufacturers are working with the FDA to set up REMs for their drugs. For example, if you plan to prescribe an ESA for a patient with cancer, you will first need to enroll in the ESA APPRISE (Assisting Providers and Cancer Patients with Risk Information for the Safe Use of ESAs) Oncology Program, which was established by ESA drug manufacturers. Once enrolled in the program, you will be assigned a unique ESA APPRISE ID number. ESA prescribers will be required to provide and review the ESA Medication Guide and counsel each patient on the risks and benefits of ESAs before each course of therapy. This counseling must be done no more than every 30 days after the first dose. (At the same time, prescribing ESAs to non-cancer patients basically only entails patient education.)

Providers also will be required to fill out the ESA APPRISE Oncology Program Patient and Healthcare Professional Acknowledgment form, which documents that the risk-benefit discussion occurred. This form is in triplicate and includes both the patient and the provider signature. One copy is given to the patient, another copy placed in the chart, and the third copy is stored for potential future audit.

For more information about the ESA APPRISE Oncology Program, please visit www.esaapprise.com/ESAAppriseUI/ESAAppriseUI/default.jsp. TH

The magnitude of medication nonadherence and the consequent negative health impact are great and should create a sense of urgency among clinicians to address this serious problem.

The magnitude of medication nonadherence and the consequent negative health impact are great and should create a sense of urgency among clinicians to address this serious problem.

The magnitude of medication nonadherence and the consequent negative health impact are great and should create a sense of urgency among clinicians to address this serious problem.

Giant cell arteritis is the most common primary systemic vasculitis. The disease occurs almost exclusively in people over age 50, with an annual incidence of 15 to 25 per 100,000.¹ Incidence rates vary significantly depending on ethnicity. The highest rates are in whites, particularly those of North European descent.² Incidence rates progressively increase after age 50. The disease is more prevalent in women. Its cause is unknown; both genetic and environmental factors are thought to play a role.

INFLAMED ARTERIES

Giant cell arteritis is characterized by a granulomatous inflammatory infiltrate affecting large and medium-size arteries. Not all vessels are equally affected: the most susceptible are the cranial arteries, the aorta, and the aorta’s primary branches, particularly those in the upper extremities.

The disease is usually associated with an intense acute-phase response. Vessel wall inflammation results in intimal hyperplasia, luminal occlusion, and tissue ischemia. Typical histologic features include a mononuclear inflammatory infiltrate primarily composed of CD4+ T cells and activated macrophages. Multinucleated giant cells are seen in only about 50% of positive biopsies; therefore, their presence is not essential for the diagnosis.³

FOUR MAIN PHENOTYPES

Some of the possible symptoms of giant cell arteritis readily point to the correct diagnosis, eg, those due to cranial artery involvement, such as temporal headache, claudication of masticatory muscles, and visual changes. However, the clinical presentation can be quite varied.

There are four predominant clinical phenotypes, which may be present at the onset of disease or appear later as the disease progresses. Although they will be described separately in this review, these clinical presentations often overlap.

Cranial arteritis

Cranial arteritis is the clinical presentation most readily associated with giant cell arteritis. Clinical features result from involvement of branches of the external or internal carotid artery.

Headache, the most frequent symptom, is typically but not exclusively localized to the temporal areas.

Visual loss is due to involvement of the branches of the ophthalmic or posterior ciliary arteries, resulting in ischemia of the optic nerve or its tracts. It occurs in up to 20% of patients.^4,5

Other symptoms and complications from cranial arteritis include tenderness of the scalp and temporal areas, claudication of the tongue or jaw muscles, stroke, and more rarely, tongue infarction.

Polymyalgia rheumatica

Polymyalgia rheumatica is a clinical syndrome that can occur by itself or in conjunction with giant cell arteritis. It may occur independently of giant cell arteritis, but also occurs in about 40% of patients with giant cell arteritis. It may precede, develop simultaneously with, or develop later during the course of the giant cell arteritis.^6,7 It is a common clinical manifestation in relapses of giant cell arteritis, even in those who did not have symptoms of polymyalgia rheumatica at the time giant cell arteritis was diagnosed.

Polymyalgia rheumatica is characterized by aching of the shoulder and hip girdle, with morning stiffness. Fatigue and malaise are often present and may be severe. Some patients with polymyalgia rheumatica may also present with peripheral joint synovitis, which may be mistakenly diagnosed as rheumatoid arthritis.⁸ Muscle weakness and elevated muscle enzymes are not associated with polymyalgia rheumatica.

Polymyalgia rheumatica is a clinical diagnosis. Approximately 80% of patients with polymyalgia rheumatica have an elevated erythrocyte sedimentation rate or an elevated C-reactive protein level.⁹ When it occurs in the absence of giant cell arteritis, it is treated differently, with less intense doses of corticosteroids. All patients with polymyalgia rheumatica should be routinely questioned regarding symptoms of giant cell arteritis.

Nonspecific systemic inflammatory disease

Some patients with giant cell arteritis may present with a nonspecific systemic inflammatory disease characterized by some combination of fever, night sweats, fatigue, malaise, and weight loss. In these patients, the diagnosis may be delayed by the lack of localizing symptoms.

Laboratory tests typically show anemia, leukocytosis, and thrombocytosis. The erythrocyte sedimentation rate and the C-reactive protein level are usually very high.

Giant cell arteritis should be in the differential diagnosis when these signs and symptoms are found in patients over age 50.

Large-vessel vasculitis

Although thoracic aortic aneurysm and dissection have been described as late complications of giant cell arteritis, large-vessel vasculitis may precede or occur concomitantly with cranial arteritis early in the disease.^10,11

Population-based surveys have shown that large-vessel vasculitis is extremely frequent in patients with giant cell arteritis. In a postmortem study of 11 patients with giant cell arteritis, all of them had evidence of arteritis involving the subclavian artery, the carotid artery, and the aorta.¹²

Patients may have no symptoms or may present with symptoms or signs of tissue ischemia such as claudication of the extremities, carotid artery tenderness, decreased or absent pulses, and large-vessel bruits on physical examination.

CONSIDER THE DIAGNOSIS IN OLDER PATIENTS

Giant cell arteritis should always be considered in patients over age 50 who have any of the clinical features described above. It is therefore very important to be familiar with its symptoms and signs.

A complete and detailed history and a detailed but focused physical examination that includes a comprehensive vascular examination are the first and most important steps in establishing the diagnosis. The vascular examination includes measuring the blood pressure in all four extremities, palpating the peripheral pulses, listening for bruits, and palpating the temporal arteries.

Temporal artery biopsy: The gold standard

Confirming the diagnosis of giant cell arteritis requires histologic findings of inflammation in the temporal artery. Superficial temporal artery biopsy is recommended for diagnostic confirmation in patients who have cranial symptoms and other signs suggesting the disease.

The biopsy should be performed on the same side as the symptoms or abnormal findings on examination. Performing a biopsy in both temporal arteries may increase the diagnostic yield but may not need to be done routinely.¹³

Although some experts recommend temporal artery biopsy in all patients in whom giant cell arteritis is suspected, biopsy has a lower diagnostic yield in patients who have no cranial symptoms. Interestingly, 5% to 15% of temporal artery biopsies performed in patients who had isolated polymyalgia rheumatica were found to be positive.^14,15 Patients with polymyalgia rheumatica and no clinical symptoms to suggest giant cell arteritis generally are not biopsied.

The segmental nature of the inflammation involving the temporal artery in giant cell arteritis may result in negative biopsy results in patients with giant cell arteritis. A temporal artery biopsy length of 5 mm or less has a very low (8%) rate of positive results, whereas a length longer than 20 mm exceeds a 50% rate of positive results. Although the optimal length of a temporal artery specimen is still debated, a longer biopsy specimen should be obtained to increase the chance of arterial specimens showing inflammatory changes.^16,17

Laboratory studies: Acute-phase reactants may be elevated

High levels of acute-phase reactants should increase one’s suspicion of giant cell arteritis. Elevations in the erythrocyte sedimentation rate and C-reactive protein and interleukin 6 levels reflect the inflammatory process in this disease.¹⁹ However, not all patients with giant cell arteritis have a high sedimentation rate, and as many as 20% of patients with biopsy-proven giant cell arteritis have a normal sedimentation rate before therapy.²⁰ Therefore, a normal sedimentation rate does not exclude the diagnosis of giant cell arteritis and should not delay its diagnosis and treatment.

As a result of systemic inflammation, the patient may also present with normochromic normocytic anemia, leukocytosis, and thrombocytosis.

Imaging studies are controversial

Imaging studies are potentially useful diagnostic tools in large-vessel vasculitis but are still the subject of significant controversy.

Ultrasonography of the temporal artery has been a controversial subject in many studies.^21,22 Color duplex ultrasonography of the temporal artery has been reported to be helpful in the diagnosis of giant cell arteritis (showing a “halo” around the arterial lumen), but further studies are needed to establish its clinical utility.

At this time, temporal artery biopsy remains the gold standard diagnostic test for giant cell arteritis, and ultrasonography is neither a substitute for biopsy nor a screening test for this disease.²³ Some have suggested, however, that ultrasonography may help to identify the best site for biopsy of the temporal artery in some patients.

Arteriography is an accurate technique for evaluating the vessel lumen and allows for measuring central blood pressure and performing vascular interventions. However, because of potential complications, it has been largely replaced by noninvasive angiographic imaging to delineate vascular anatomy.

TREATMENT

Glucocorticoid therapy remains the standard of care

Once the diagnosis of giant cell arteritis is established, glucocorticoid treatment should be started. Glucocorticoids are the standard therapy, and they usually bring about a prompt clinical response. Although never evaluated in placebo-controlled trials, these drugs have been shown to prevent progression of visual loss in a retrospective study.²⁶

In patients with visual symptoms or imminent visual loss, therapy should be started promptly once suspicion of giant cell arteritis is raised; ie, one should not wait until the diagnosis is confirmed by biopsy.

Ideally, a glucocorticoid should be started after a temporal artery biopsy is done, but treatment should not be delayed, as it rapidly suppresses the inflammatory response and may prevent complications from tissue ischemia, such as blindness. Visual loss is usually irreversible.

There is still a role for obtaining a temporal artery biopsy up to several weeks after glucocorticoid therapy is started, as the pathological abnormalities of arteritis do not rapidly resolve.²⁷

Glucocorticoid therapy is highly effective in inducing disease remission in patients with giant cell arteritis. Nearly all patients respond to 1 mg/kg (40–60 mg) per day of prednisone or its equivalent.

The initial dosing is usually maintained for 4 weeks and then decreased slowly. The duration of therapy varies; most patients remain on therapy for at least 1 year, and some cannot stop it completely without recurrence of symptoms.

If a patient is about to lose his or her vision or has lost all or some vision in one eye, a higher initial dose of a glucocorticoid is usually used (ie, a pulse of 500 or 1,000 mg of intravenous methylprednisolone) and may be beneficial.²⁸

Although a rapid clinical response to therapy is usually seen within 48 hours, some patients may have a more delayed clinical improvement.

Alternate-day therapy was compared with daily therapy and was found to be less effective, and as a result it is not recommended.²⁹

Glucocorticoid therapy can cause significant toxicity in patients with giant cell arteritis, as they commonly must take these drugs for long periods. The rate of relapse in those who discontinue therapy is quite high—as high as 77% within 12 months.³⁰

Given the concern about glucocorticoid toxicity, several studies have evaluated alternative strategies and other immunosuppressive drugs. However, no study has concluded that other medications are effective in the treatment of giant cell arteritis.

Mazlumzadeh et al³¹ evaluated the initial use of intravenous pulse methylprednisolone therapy (15 mg/kg ideal body weight on 3 consecutive days) in an attempt to decrease the glucocorticoid requirement. Although the group receiving this therapy had a lower relapse rate than in the placebo group, and their cumulative dose of glucocorticoid was lower (all patients also received oral prednisone), there was no reduction in the rate of glucocorticoid-associated toxicity.³¹ Care must be taken to prevent and monitor for corticosteroid complications such as osteoporosis, glaucoma, diabetes mellitus, and hypertension.

Methotrexate: Mixed results in clinical trials

Methotrexate has been evaluated in three prospective randomized trials,^30,32,33 with mixed results.

Spiera et al³² enrolled 21 patients in a double-blind placebo-controlled trial: 12 patients received low-dose methotrexate (7.5 mg/week) and 9 received placebo. In addition, all 21 received a glucocorticoid. There was no significant difference between the methotrexate- and placebo-treated patients in the cumulative dose of glucocorticoid, duration of glucocorticoid therapy, time to taper off the glucocorticoid to less than 10 mg of prednisone per day, or glucocorticoidrelated adverse effects.

Jover et al,³³ in another double-blind placebo-controlled trial, studied 42 patients with giant cell arteritis, half of whom were randomized to receive methotrexate 10 mg/week, while the other half received placebo. All patients received prednisone. Patients in the methotrexate group had fewer relapses and a 25% lower cumulative dose of prednisone during follow-up. However, the incidence of adverse events was similar in both groups. Methotrexate was discontinued in 3 patients who developed drug-related adverse events.

Hoffman et al³⁰ randomized 98 patients to receive either methotrexate (up to 15 mg/week) or placebo in a double-blind fashion. All patients also received prednisone at an initial dose of 1 mg/kg/day (up to 60 mg/day). At completion of the study, no differences between the groups were noted in the rates of relapse or treatment-related morbidity or in the cumulative dose of glucocorticoid. However, treatment with methotrexate appeared to lower the rate of recurrence of isolated polymyalgia rheumatica in a small number of patients.³⁰

Comment. Differences in the results of these trials may be attributed to several factors, including different definitions of relapses and different glucocorticoid doses and tapering regimens.

A meta-analysis of these three trials³⁴ showed a reduction in the risk of relapse: 4 patients would have to be treated to prevent one first relapse, 5 would have to be treated to prevent one second relapse, and 11 would need to be treated to prevent one first relapse of cranial symptoms in the first 48 weeks. However, the main goal of methotrexate therapy is to decrease the frequency of adverse events from glucocorticoids, and this meta-analysis found no difference in rates of glucocorticoid-related adverse events in patients treated with methotrexate.

The study raises the question of whether methotrexate should be further evaluated in in different patient populations and at higher doses.³⁴

Infliximab is not recommended

In a prospective study, patients with giant cell arteritis were randomly assigned to receive either infliximab (Remicade) 5 mg/kg every 8 weeks or placebo, in addition to standard glucocorticoid therapy. The study showed no significant difference in the relapse rate and a higher rate of infection in the infliximab group (71%) than in the placebo group (56%). Given the lack of any benefit observed in this study, infliximab is not recommended in the treatment of patients with giant cell arteritis.³⁵

Aspirin is recommended

Daily low-dose aspirin therapy has been shown in several studies to be effective in preventing ischemic complications of giant cell arteritis, including stroke and visual loss. It is currently recommended that all patients with giant cell arteritis without a major contraindication take aspirin 81 mg daily.^36–38

Giant cell arteritis is the most common primary systemic vasculitis. The disease occurs almost exclusively in people over age 50, with an annual incidence of 15 to 25 per 100,000.¹ Incidence rates vary significantly depending on ethnicity. The highest rates are in whites, particularly those of North European descent.² Incidence rates progressively increase after age 50. The disease is more prevalent in women. Its cause is unknown; both genetic and environmental factors are thought to play a role.

INFLAMED ARTERIES

Giant cell arteritis is characterized by a granulomatous inflammatory infiltrate affecting large and medium-size arteries. Not all vessels are equally affected: the most susceptible are the cranial arteries, the aorta, and the aorta’s primary branches, particularly those in the upper extremities.

The disease is usually associated with an intense acute-phase response. Vessel wall inflammation results in intimal hyperplasia, luminal occlusion, and tissue ischemia. Typical histologic features include a mononuclear inflammatory infiltrate primarily composed of CD4+ T cells and activated macrophages. Multinucleated giant cells are seen in only about 50% of positive biopsies; therefore, their presence is not essential for the diagnosis.³

FOUR MAIN PHENOTYPES

Some of the possible symptoms of giant cell arteritis readily point to the correct diagnosis, eg, those due to cranial artery involvement, such as temporal headache, claudication of masticatory muscles, and visual changes. However, the clinical presentation can be quite varied.

There are four predominant clinical phenotypes, which may be present at the onset of disease or appear later as the disease progresses. Although they will be described separately in this review, these clinical presentations often overlap.

Cranial arteritis

Cranial arteritis is the clinical presentation most readily associated with giant cell arteritis. Clinical features result from involvement of branches of the external or internal carotid artery.

Headache, the most frequent symptom, is typically but not exclusively localized to the temporal areas.

Visual loss is due to involvement of the branches of the ophthalmic or posterior ciliary arteries, resulting in ischemia of the optic nerve or its tracts. It occurs in up to 20% of patients.^4,5

Other symptoms and complications from cranial arteritis include tenderness of the scalp and temporal areas, claudication of the tongue or jaw muscles, stroke, and more rarely, tongue infarction.

Polymyalgia rheumatica

Polymyalgia rheumatica is a clinical syndrome that can occur by itself or in conjunction with giant cell arteritis. It may occur independently of giant cell arteritis, but also occurs in about 40% of patients with giant cell arteritis. It may precede, develop simultaneously with, or develop later during the course of the giant cell arteritis.^6,7 It is a common clinical manifestation in relapses of giant cell arteritis, even in those who did not have symptoms of polymyalgia rheumatica at the time giant cell arteritis was diagnosed.

Polymyalgia rheumatica is characterized by aching of the shoulder and hip girdle, with morning stiffness. Fatigue and malaise are often present and may be severe. Some patients with polymyalgia rheumatica may also present with peripheral joint synovitis, which may be mistakenly diagnosed as rheumatoid arthritis.⁸ Muscle weakness and elevated muscle enzymes are not associated with polymyalgia rheumatica.

Polymyalgia rheumatica is a clinical diagnosis. Approximately 80% of patients with polymyalgia rheumatica have an elevated erythrocyte sedimentation rate or an elevated C-reactive protein level.⁹ When it occurs in the absence of giant cell arteritis, it is treated differently, with less intense doses of corticosteroids. All patients with polymyalgia rheumatica should be routinely questioned regarding symptoms of giant cell arteritis.

Nonspecific systemic inflammatory disease

Some patients with giant cell arteritis may present with a nonspecific systemic inflammatory disease characterized by some combination of fever, night sweats, fatigue, malaise, and weight loss. In these patients, the diagnosis may be delayed by the lack of localizing symptoms.

Laboratory tests typically show anemia, leukocytosis, and thrombocytosis. The erythrocyte sedimentation rate and the C-reactive protein level are usually very high.

Giant cell arteritis should be in the differential diagnosis when these signs and symptoms are found in patients over age 50.

Large-vessel vasculitis

Although thoracic aortic aneurysm and dissection have been described as late complications of giant cell arteritis, large-vessel vasculitis may precede or occur concomitantly with cranial arteritis early in the disease.^10,11

Population-based surveys have shown that large-vessel vasculitis is extremely frequent in patients with giant cell arteritis. In a postmortem study of 11 patients with giant cell arteritis, all of them had evidence of arteritis involving the subclavian artery, the carotid artery, and the aorta.¹²

Patients may have no symptoms or may present with symptoms or signs of tissue ischemia such as claudication of the extremities, carotid artery tenderness, decreased or absent pulses, and large-vessel bruits on physical examination.

CONSIDER THE DIAGNOSIS IN OLDER PATIENTS

Giant cell arteritis should always be considered in patients over age 50 who have any of the clinical features described above. It is therefore very important to be familiar with its symptoms and signs.

A complete and detailed history and a detailed but focused physical examination that includes a comprehensive vascular examination are the first and most important steps in establishing the diagnosis. The vascular examination includes measuring the blood pressure in all four extremities, palpating the peripheral pulses, listening for bruits, and palpating the temporal arteries.

Temporal artery biopsy: The gold standard

Confirming the diagnosis of giant cell arteritis requires histologic findings of inflammation in the temporal artery. Superficial temporal artery biopsy is recommended for diagnostic confirmation in patients who have cranial symptoms and other signs suggesting the disease.

The biopsy should be performed on the same side as the symptoms or abnormal findings on examination. Performing a biopsy in both temporal arteries may increase the diagnostic yield but may not need to be done routinely.¹³

Although some experts recommend temporal artery biopsy in all patients in whom giant cell arteritis is suspected, biopsy has a lower diagnostic yield in patients who have no cranial symptoms. Interestingly, 5% to 15% of temporal artery biopsies performed in patients who had isolated polymyalgia rheumatica were found to be positive.^14,15 Patients with polymyalgia rheumatica and no clinical symptoms to suggest giant cell arteritis generally are not biopsied.

The segmental nature of the inflammation involving the temporal artery in giant cell arteritis may result in negative biopsy results in patients with giant cell arteritis. A temporal artery biopsy length of 5 mm or less has a very low (8%) rate of positive results, whereas a length longer than 20 mm exceeds a 50% rate of positive results. Although the optimal length of a temporal artery specimen is still debated, a longer biopsy specimen should be obtained to increase the chance of arterial specimens showing inflammatory changes.^16,17

Laboratory studies: Acute-phase reactants may be elevated

High levels of acute-phase reactants should increase one’s suspicion of giant cell arteritis. Elevations in the erythrocyte sedimentation rate and C-reactive protein and interleukin 6 levels reflect the inflammatory process in this disease.¹⁹ However, not all patients with giant cell arteritis have a high sedimentation rate, and as many as 20% of patients with biopsy-proven giant cell arteritis have a normal sedimentation rate before therapy.²⁰ Therefore, a normal sedimentation rate does not exclude the diagnosis of giant cell arteritis and should not delay its diagnosis and treatment.

As a result of systemic inflammation, the patient may also present with normochromic normocytic anemia, leukocytosis, and thrombocytosis.

Imaging studies are controversial

Imaging studies are potentially useful diagnostic tools in large-vessel vasculitis but are still the subject of significant controversy.

Ultrasonography of the temporal artery has been a controversial subject in many studies.^21,22 Color duplex ultrasonography of the temporal artery has been reported to be helpful in the diagnosis of giant cell arteritis (showing a “halo” around the arterial lumen), but further studies are needed to establish its clinical utility.

At this time, temporal artery biopsy remains the gold standard diagnostic test for giant cell arteritis, and ultrasonography is neither a substitute for biopsy nor a screening test for this disease.²³ Some have suggested, however, that ultrasonography may help to identify the best site for biopsy of the temporal artery in some patients.

Arteriography is an accurate technique for evaluating the vessel lumen and allows for measuring central blood pressure and performing vascular interventions. However, because of potential complications, it has been largely replaced by noninvasive angiographic imaging to delineate vascular anatomy.

TREATMENT

Glucocorticoid therapy remains the standard of care

Once the diagnosis of giant cell arteritis is established, glucocorticoid treatment should be started. Glucocorticoids are the standard therapy, and they usually bring about a prompt clinical response. Although never evaluated in placebo-controlled trials, these drugs have been shown to prevent progression of visual loss in a retrospective study.²⁶

In patients with visual symptoms or imminent visual loss, therapy should be started promptly once suspicion of giant cell arteritis is raised; ie, one should not wait until the diagnosis is confirmed by biopsy.

Ideally, a glucocorticoid should be started after a temporal artery biopsy is done, but treatment should not be delayed, as it rapidly suppresses the inflammatory response and may prevent complications from tissue ischemia, such as blindness. Visual loss is usually irreversible.

There is still a role for obtaining a temporal artery biopsy up to several weeks after glucocorticoid therapy is started, as the pathological abnormalities of arteritis do not rapidly resolve.²⁷

Glucocorticoid therapy is highly effective in inducing disease remission in patients with giant cell arteritis. Nearly all patients respond to 1 mg/kg (40–60 mg) per day of prednisone or its equivalent.

The initial dosing is usually maintained for 4 weeks and then decreased slowly. The duration of therapy varies; most patients remain on therapy for at least 1 year, and some cannot stop it completely without recurrence of symptoms.

If a patient is about to lose his or her vision or has lost all or some vision in one eye, a higher initial dose of a glucocorticoid is usually used (ie, a pulse of 500 or 1,000 mg of intravenous methylprednisolone) and may be beneficial.²⁸

Although a rapid clinical response to therapy is usually seen within 48 hours, some patients may have a more delayed clinical improvement.

Alternate-day therapy was compared with daily therapy and was found to be less effective, and as a result it is not recommended.²⁹

Glucocorticoid therapy can cause significant toxicity in patients with giant cell arteritis, as they commonly must take these drugs for long periods. The rate of relapse in those who discontinue therapy is quite high—as high as 77% within 12 months.³⁰

Given the concern about glucocorticoid toxicity, several studies have evaluated alternative strategies and other immunosuppressive drugs. However, no study has concluded that other medications are effective in the treatment of giant cell arteritis.

Mazlumzadeh et al³¹ evaluated the initial use of intravenous pulse methylprednisolone therapy (15 mg/kg ideal body weight on 3 consecutive days) in an attempt to decrease the glucocorticoid requirement. Although the group receiving this therapy had a lower relapse rate than in the placebo group, and their cumulative dose of glucocorticoid was lower (all patients also received oral prednisone), there was no reduction in the rate of glucocorticoid-associated toxicity.³¹ Care must be taken to prevent and monitor for corticosteroid complications such as osteoporosis, glaucoma, diabetes mellitus, and hypertension.

Methotrexate: Mixed results in clinical trials

Methotrexate has been evaluated in three prospective randomized trials,^30,32,33 with mixed results.

Spiera et al³² enrolled 21 patients in a double-blind placebo-controlled trial: 12 patients received low-dose methotrexate (7.5 mg/week) and 9 received placebo. In addition, all 21 received a glucocorticoid. There was no significant difference between the methotrexate- and placebo-treated patients in the cumulative dose of glucocorticoid, duration of glucocorticoid therapy, time to taper off the glucocorticoid to less than 10 mg of prednisone per day, or glucocorticoidrelated adverse effects.

Jover et al,³³ in another double-blind placebo-controlled trial, studied 42 patients with giant cell arteritis, half of whom were randomized to receive methotrexate 10 mg/week, while the other half received placebo. All patients received prednisone. Patients in the methotrexate group had fewer relapses and a 25% lower cumulative dose of prednisone during follow-up. However, the incidence of adverse events was similar in both groups. Methotrexate was discontinued in 3 patients who developed drug-related adverse events.

Hoffman et al³⁰ randomized 98 patients to receive either methotrexate (up to 15 mg/week) or placebo in a double-blind fashion. All patients also received prednisone at an initial dose of 1 mg/kg/day (up to 60 mg/day). At completion of the study, no differences between the groups were noted in the rates of relapse or treatment-related morbidity or in the cumulative dose of glucocorticoid. However, treatment with methotrexate appeared to lower the rate of recurrence of isolated polymyalgia rheumatica in a small number of patients.³⁰

Comment. Differences in the results of these trials may be attributed to several factors, including different definitions of relapses and different glucocorticoid doses and tapering regimens.

A meta-analysis of these three trials³⁴ showed a reduction in the risk of relapse: 4 patients would have to be treated to prevent one first relapse, 5 would have to be treated to prevent one second relapse, and 11 would need to be treated to prevent one first relapse of cranial symptoms in the first 48 weeks. However, the main goal of methotrexate therapy is to decrease the frequency of adverse events from glucocorticoids, and this meta-analysis found no difference in rates of glucocorticoid-related adverse events in patients treated with methotrexate.

The study raises the question of whether methotrexate should be further evaluated in in different patient populations and at higher doses.³⁴

Infliximab is not recommended

In a prospective study, patients with giant cell arteritis were randomly assigned to receive either infliximab (Remicade) 5 mg/kg every 8 weeks or placebo, in addition to standard glucocorticoid therapy. The study showed no significant difference in the relapse rate and a higher rate of infection in the infliximab group (71%) than in the placebo group (56%). Given the lack of any benefit observed in this study, infliximab is not recommended in the treatment of patients with giant cell arteritis.³⁵

Aspirin is recommended

Daily low-dose aspirin therapy has been shown in several studies to be effective in preventing ischemic complications of giant cell arteritis, including stroke and visual loss. It is currently recommended that all patients with giant cell arteritis without a major contraindication take aspirin 81 mg daily.^36–38

Giant cell arteritis is the most common primary systemic vasculitis. The disease occurs almost exclusively in people over age 50, with an annual incidence of 15 to 25 per 100,000.¹ Incidence rates vary significantly depending on ethnicity. The highest rates are in whites, particularly those of North European descent.² Incidence rates progressively increase after age 50. The disease is more prevalent in women. Its cause is unknown; both genetic and environmental factors are thought to play a role.

INFLAMED ARTERIES

Giant cell arteritis is characterized by a granulomatous inflammatory infiltrate affecting large and medium-size arteries. Not all vessels are equally affected: the most susceptible are the cranial arteries, the aorta, and the aorta’s primary branches, particularly those in the upper extremities.

The disease is usually associated with an intense acute-phase response. Vessel wall inflammation results in intimal hyperplasia, luminal occlusion, and tissue ischemia. Typical histologic features include a mononuclear inflammatory infiltrate primarily composed of CD4+ T cells and activated macrophages. Multinucleated giant cells are seen in only about 50% of positive biopsies; therefore, their presence is not essential for the diagnosis.³

FOUR MAIN PHENOTYPES

Some of the possible symptoms of giant cell arteritis readily point to the correct diagnosis, eg, those due to cranial artery involvement, such as temporal headache, claudication of masticatory muscles, and visual changes. However, the clinical presentation can be quite varied.

There are four predominant clinical phenotypes, which may be present at the onset of disease or appear later as the disease progresses. Although they will be described separately in this review, these clinical presentations often overlap.

Cranial arteritis

Cranial arteritis is the clinical presentation most readily associated with giant cell arteritis. Clinical features result from involvement of branches of the external or internal carotid artery.

Headache, the most frequent symptom, is typically but not exclusively localized to the temporal areas.

Visual loss is due to involvement of the branches of the ophthalmic or posterior ciliary arteries, resulting in ischemia of the optic nerve or its tracts. It occurs in up to 20% of patients.^4,5

Other symptoms and complications from cranial arteritis include tenderness of the scalp and temporal areas, claudication of the tongue or jaw muscles, stroke, and more rarely, tongue infarction.

Polymyalgia rheumatica

Polymyalgia rheumatica is a clinical syndrome that can occur by itself or in conjunction with giant cell arteritis. It may occur independently of giant cell arteritis, but also occurs in about 40% of patients with giant cell arteritis. It may precede, develop simultaneously with, or develop later during the course of the giant cell arteritis.^6,7 It is a common clinical manifestation in relapses of giant cell arteritis, even in those who did not have symptoms of polymyalgia rheumatica at the time giant cell arteritis was diagnosed.

Polymyalgia rheumatica is characterized by aching of the shoulder and hip girdle, with morning stiffness. Fatigue and malaise are often present and may be severe. Some patients with polymyalgia rheumatica may also present with peripheral joint synovitis, which may be mistakenly diagnosed as rheumatoid arthritis.⁸ Muscle weakness and elevated muscle enzymes are not associated with polymyalgia rheumatica.

Polymyalgia rheumatica is a clinical diagnosis. Approximately 80% of patients with polymyalgia rheumatica have an elevated erythrocyte sedimentation rate or an elevated C-reactive protein level.⁹ When it occurs in the absence of giant cell arteritis, it is treated differently, with less intense doses of corticosteroids. All patients with polymyalgia rheumatica should be routinely questioned regarding symptoms of giant cell arteritis.

Nonspecific systemic inflammatory disease

Some patients with giant cell arteritis may present with a nonspecific systemic inflammatory disease characterized by some combination of fever, night sweats, fatigue, malaise, and weight loss. In these patients, the diagnosis may be delayed by the lack of localizing symptoms.

Laboratory tests typically show anemia, leukocytosis, and thrombocytosis. The erythrocyte sedimentation rate and the C-reactive protein level are usually very high.

Giant cell arteritis should be in the differential diagnosis when these signs and symptoms are found in patients over age 50.

Large-vessel vasculitis

Although thoracic aortic aneurysm and dissection have been described as late complications of giant cell arteritis, large-vessel vasculitis may precede or occur concomitantly with cranial arteritis early in the disease.^10,11

Population-based surveys have shown that large-vessel vasculitis is extremely frequent in patients with giant cell arteritis. In a postmortem study of 11 patients with giant cell arteritis, all of them had evidence of arteritis involving the subclavian artery, the carotid artery, and the aorta.¹²

Patients may have no symptoms or may present with symptoms or signs of tissue ischemia such as claudication of the extremities, carotid artery tenderness, decreased or absent pulses, and large-vessel bruits on physical examination.

CONSIDER THE DIAGNOSIS IN OLDER PATIENTS

Giant cell arteritis should always be considered in patients over age 50 who have any of the clinical features described above. It is therefore very important to be familiar with its symptoms and signs.

A complete and detailed history and a detailed but focused physical examination that includes a comprehensive vascular examination are the first and most important steps in establishing the diagnosis. The vascular examination includes measuring the blood pressure in all four extremities, palpating the peripheral pulses, listening for bruits, and palpating the temporal arteries.

Temporal artery biopsy: The gold standard

Confirming the diagnosis of giant cell arteritis requires histologic findings of inflammation in the temporal artery. Superficial temporal artery biopsy is recommended for diagnostic confirmation in patients who have cranial symptoms and other signs suggesting the disease.

The biopsy should be performed on the same side as the symptoms or abnormal findings on examination. Performing a biopsy in both temporal arteries may increase the diagnostic yield but may not need to be done routinely.¹³

Although some experts recommend temporal artery biopsy in all patients in whom giant cell arteritis is suspected, biopsy has a lower diagnostic yield in patients who have no cranial symptoms. Interestingly, 5% to 15% of temporal artery biopsies performed in patients who had isolated polymyalgia rheumatica were found to be positive.^14,15 Patients with polymyalgia rheumatica and no clinical symptoms to suggest giant cell arteritis generally are not biopsied.

The segmental nature of the inflammation involving the temporal artery in giant cell arteritis may result in negative biopsy results in patients with giant cell arteritis. A temporal artery biopsy length of 5 mm or less has a very low (8%) rate of positive results, whereas a length longer than 20 mm exceeds a 50% rate of positive results. Although the optimal length of a temporal artery specimen is still debated, a longer biopsy specimen should be obtained to increase the chance of arterial specimens showing inflammatory changes.^16,17

Laboratory studies: Acute-phase reactants may be elevated

High levels of acute-phase reactants should increase one’s suspicion of giant cell arteritis. Elevations in the erythrocyte sedimentation rate and C-reactive protein and interleukin 6 levels reflect the inflammatory process in this disease.¹⁹ However, not all patients with giant cell arteritis have a high sedimentation rate, and as many as 20% of patients with biopsy-proven giant cell arteritis have a normal sedimentation rate before therapy.²⁰ Therefore, a normal sedimentation rate does not exclude the diagnosis of giant cell arteritis and should not delay its diagnosis and treatment.

As a result of systemic inflammation, the patient may also present with normochromic normocytic anemia, leukocytosis, and thrombocytosis.

Imaging studies are controversial

Imaging studies are potentially useful diagnostic tools in large-vessel vasculitis but are still the subject of significant controversy.

Ultrasonography of the temporal artery has been a controversial subject in many studies.^21,22 Color duplex ultrasonography of the temporal artery has been reported to be helpful in the diagnosis of giant cell arteritis (showing a “halo” around the arterial lumen), but further studies are needed to establish its clinical utility.

At this time, temporal artery biopsy remains the gold standard diagnostic test for giant cell arteritis, and ultrasonography is neither a substitute for biopsy nor a screening test for this disease.²³ Some have suggested, however, that ultrasonography may help to identify the best site for biopsy of the temporal artery in some patients.

Arteriography is an accurate technique for evaluating the vessel lumen and allows for measuring central blood pressure and performing vascular interventions. However, because of potential complications, it has been largely replaced by noninvasive angiographic imaging to delineate vascular anatomy.

TREATMENT

Glucocorticoid therapy remains the standard of care

Once the diagnosis of giant cell arteritis is established, glucocorticoid treatment should be started. Glucocorticoids are the standard therapy, and they usually bring about a prompt clinical response. Although never evaluated in placebo-controlled trials, these drugs have been shown to prevent progression of visual loss in a retrospective study.²⁶

In patients with visual symptoms or imminent visual loss, therapy should be started promptly once suspicion of giant cell arteritis is raised; ie, one should not wait until the diagnosis is confirmed by biopsy.

Ideally, a glucocorticoid should be started after a temporal artery biopsy is done, but treatment should not be delayed, as it rapidly suppresses the inflammatory response and may prevent complications from tissue ischemia, such as blindness. Visual loss is usually irreversible.

There is still a role for obtaining a temporal artery biopsy up to several weeks after glucocorticoid therapy is started, as the pathological abnormalities of arteritis do not rapidly resolve.²⁷

Glucocorticoid therapy is highly effective in inducing disease remission in patients with giant cell arteritis. Nearly all patients respond to 1 mg/kg (40–60 mg) per day of prednisone or its equivalent.

The initial dosing is usually maintained for 4 weeks and then decreased slowly. The duration of therapy varies; most patients remain on therapy for at least 1 year, and some cannot stop it completely without recurrence of symptoms.

If a patient is about to lose his or her vision or has lost all or some vision in one eye, a higher initial dose of a glucocorticoid is usually used (ie, a pulse of 500 or 1,000 mg of intravenous methylprednisolone) and may be beneficial.²⁸

Although a rapid clinical response to therapy is usually seen within 48 hours, some patients may have a more delayed clinical improvement.

Alternate-day therapy was compared with daily therapy and was found to be less effective, and as a result it is not recommended.²⁹

Glucocorticoid therapy can cause significant toxicity in patients with giant cell arteritis, as they commonly must take these drugs for long periods. The rate of relapse in those who discontinue therapy is quite high—as high as 77% within 12 months.³⁰

Given the concern about glucocorticoid toxicity, several studies have evaluated alternative strategies and other immunosuppressive drugs. However, no study has concluded that other medications are effective in the treatment of giant cell arteritis.

Mazlumzadeh et al³¹ evaluated the initial use of intravenous pulse methylprednisolone therapy (15 mg/kg ideal body weight on 3 consecutive days) in an attempt to decrease the glucocorticoid requirement. Although the group receiving this therapy had a lower relapse rate than in the placebo group, and their cumulative dose of glucocorticoid was lower (all patients also received oral prednisone), there was no reduction in the rate of glucocorticoid-associated toxicity.³¹ Care must be taken to prevent and monitor for corticosteroid complications such as osteoporosis, glaucoma, diabetes mellitus, and hypertension.

Methotrexate: Mixed results in clinical trials

Methotrexate has been evaluated in three prospective randomized trials,^30,32,33 with mixed results.

Spiera et al³² enrolled 21 patients in a double-blind placebo-controlled trial: 12 patients received low-dose methotrexate (7.5 mg/week) and 9 received placebo. In addition, all 21 received a glucocorticoid. There was no significant difference between the methotrexate- and placebo-treated patients in the cumulative dose of glucocorticoid, duration of glucocorticoid therapy, time to taper off the glucocorticoid to less than 10 mg of prednisone per day, or glucocorticoidrelated adverse effects.

Jover et al,³³ in another double-blind placebo-controlled trial, studied 42 patients with giant cell arteritis, half of whom were randomized to receive methotrexate 10 mg/week, while the other half received placebo. All patients received prednisone. Patients in the methotrexate group had fewer relapses and a 25% lower cumulative dose of prednisone during follow-up. However, the incidence of adverse events was similar in both groups. Methotrexate was discontinued in 3 patients who developed drug-related adverse events.

Hoffman et al³⁰ randomized 98 patients to receive either methotrexate (up to 15 mg/week) or placebo in a double-blind fashion. All patients also received prednisone at an initial dose of 1 mg/kg/day (up to 60 mg/day). At completion of the study, no differences between the groups were noted in the rates of relapse or treatment-related morbidity or in the cumulative dose of glucocorticoid. However, treatment with methotrexate appeared to lower the rate of recurrence of isolated polymyalgia rheumatica in a small number of patients.³⁰

Comment. Differences in the results of these trials may be attributed to several factors, including different definitions of relapses and different glucocorticoid doses and tapering regimens.

A meta-analysis of these three trials³⁴ showed a reduction in the risk of relapse: 4 patients would have to be treated to prevent one first relapse, 5 would have to be treated to prevent one second relapse, and 11 would need to be treated to prevent one first relapse of cranial symptoms in the first 48 weeks. However, the main goal of methotrexate therapy is to decrease the frequency of adverse events from glucocorticoids, and this meta-analysis found no difference in rates of glucocorticoid-related adverse events in patients treated with methotrexate.

The study raises the question of whether methotrexate should be further evaluated in in different patient populations and at higher doses.³⁴

Infliximab is not recommended

In a prospective study, patients with giant cell arteritis were randomly assigned to receive either infliximab (Remicade) 5 mg/kg every 8 weeks or placebo, in addition to standard glucocorticoid therapy. The study showed no significant difference in the relapse rate and a higher rate of infection in the infliximab group (71%) than in the placebo group (56%). Given the lack of any benefit observed in this study, infliximab is not recommended in the treatment of patients with giant cell arteritis.³⁵

Aspirin is recommended

Daily low-dose aspirin therapy has been shown in several studies to be effective in preventing ischemic complications of giant cell arteritis, including stroke and visual loss. It is currently recommended that all patients with giant cell arteritis without a major contraindication take aspirin 81 mg daily.^36–38

A 50-year-old woman presents to the emergency department because of repeated episodes of vomiting over the past 12 hours. She reports eight episodes of non-bloody, nonbilious emesis associated with palpitations and feelings of anxiety, but with no fever or diarrhea. She has not traveled recently and does not have any sick contacts.

She reports that she never had health problems until 6 months ago, when she began having panic attacks that woke her from sleep. The episodes first occurred once or twice per week, usually at night, and involved palpitations and feelings of anxiety that lasted 2 to 4 hours, but no other associated symptoms. After a month, the episodes began to occur more regularly during the day and were accompanied by a pounding headache that began in the back of her neck and extended up and over her head. Her primary care physician prescribed sertraline (Zoloft) and referred her to a neurologist to evaluate the headaches. The neurologic workup included brain magnetic resonance imaging and electroencephalography, both of which were normal.

After 8 weeks on sertraline, the episodes continued to increase in frequency and severity, and her physician switched her to paroxetine (Paxil) and added lorazepam (Ativan), which did not improve her symptoms. Over the past 2 months, during which time she has not been taking any medications, the episodes began to involve nausea and, more recently, vomiting, with episodes occurring as often as once or twice daily, and with intermittent symptom-free days. None of the prior episodes was accompanied by symptoms as severe as those she is currently experiencing.

She is otherwise healthy with no chronic diseases. Her surgical history includes resection of an angiolipoma from her right arm and dilation and curettage for endometrial polyps. She has no personal or family history of psychiatric illness.

PHYSICAL EXAMINATION

The patient is slender and tremulous but does not appear diaphoretic. Her blood pressure is 176/92 mm Hg, pulse 98, temperature 36.5°C (97.7°F), and respiratory rate 20 per minute. Oxygen saturation by pulse oximetry is 98% on room air. She has dry mucus membranes and orthostatic hypotension, but her physical examination is otherwise normal. Electrocardiography (ECG) shows a normal sinus rhythm with a prolonged QTc of 571 ms and peaked P and T waves.

LABORATORY VALUES

• Hemoglobin 15.6 g/dL (reference range 11.5–15.5)
• Hematocrit 47.2% (36.0–46.0)
• Platelet count 448 × 10⁹/L (150–400)
• White cell count 18.65 × 10⁹/L (3.70–11.00)
• Potassium 2.5 mmol/L (3.5–4.0)
• Chloride 97 mmol/L (98–110)
• Bicarbonate 21 mmol/L (23–32)
• Anion gap 20 mmol/L (0–15)
• Glucose 233 mg/dL (65–100).

Sodium, blood urea nitrogen, and creatinine levels are all within normal limits. Urinalysis suggests a urinary tract infection.

IS THIS A PANIC ATTACK?

1. Which of the following is not characteristic of a panic attack?

• Nausea and vomiting
• Onset during sleep
• Palpitations
• Chest pain or discomfort
• Headache
• Trembling or shaking

According to the Diagnostic and Statistical Manual of Mental Disorders (Fourth Edition) (DSM-IV), the diagnosis of panic attack requires the presence of intense fear or discomfort and four or more other symptoms that may come from any of six domains:

• Cardiovascular: palpitations, pounding heart, tachycardia, and chest pain or discomfort
• Autonomic: sweating, chills or hot flushes, and trembling or shaking
• Pulmonary: shortness of breath or a smothering sensation
• Neurologic: dizziness or light-headedness and paresthesias
• Gastrointestinal: choking and nausea or abdominal distress
• Psychological: compass derealization, depersonalization, and the fear of losing control or “going crazy.”¹

Two aspects of the patient’s history may be misinterpreted by those unfamiliar with the symptomatology of panic attack. First, although panic disorder carries an increased risk of many comorbidities, including migraine, headache is not typically associated with the panic attacks themselves.² Second, while not a part of the diagnostic criteria, sleep disturbances are common in patients with panic disorder, and 30% to 45% of patients with the disorder experience recurrent nocturnal panic attacks.³ Therefore, the correct answer is headache.

THE DIFFERENTIAL DIAGNOSIS

When considering a diagnosis of panic attack or panic disorder, the DSM-IV mandates that medical causes of the symptoms must be excluded. Common conditions causing a similar spectrum of symptoms include hyperthyroidism, caffeine and stimulant use or abuse, asthma, cardiac arrhythmias, alcohol withdrawal, and, more rarely, complex partial seizures and pheochromocytoma.^2,4 Many of these conditions can be ruled out by the history alone in a reliable patient.

Our patient’s electrocardiogram showed no evidence of ischemia or arrhythmias. Also, her recent negative neurologic workup makes seizure activity less likely.

Many of this patient’s laboratory abnormalities are easily explained by her repeated bouts of vomiting. Specifically, her elevated hemoglobin level and hematocrit are likely secondary to volume contraction, while hypochloremia is seen following losses of HCl with emesis. Typically, however, patients with vomiting have a hypochloremic metabolic alkalosis, and her low serum bicarbonate level is inconsistent with the history.

Three factors might be contributing to this patient’s hypokalemia. First, in a volume-depleted state, the cortical collecting tubules secrete potassium in exchange for increased sodium reabsorption in an attempt to correct volume status. Second, the alkalotic state caused by losses of acid with vomiting results in a transcellular shift of potassium ions into cells in exchange for hydrogen ions. Third, increased levels of epinephrine also cause a shift of potassium ions into cells.⁵ Potassium is not lost directly through nausea and vomiting.

A state of catecholamine excess, such as during a severe panic attack or in the presence of a catecholamine-secreting tumor, could explain many of her abnormalities. In addition to causing hypokalemia, epinephrine has a gluconeogenic effect, whereas norepinephrine inhibits insulin release, providing a potential explanation for hyperglycemia in a patient with no risk factors for diabetes. Finally, catecholamine excess contributes to lactic acidosis, which could help to explain the low serum bicarbonate level and the elevated anion gap, but unless we take arterial blood gas measurements, the patient’s acid-base status cannot be determined.

While panic attacks do stimulate the sympathetic nervous system, certain elements of her history raise the clinical suspicion for another process. First, the severity of the electrolyte abnormalities is suspicious. Second, a typical panic attack peaks at 10 minutes and begins to subside, whereas this woman’s symptoms have persisted for 12 hours. Finally, the clinical history, in particular the prominence of headaches associated with the symptoms, is inconsistent with classic panic attack. Consequently, an alternative diagnosis, such as pheochromocytoma, deserves more careful evaluation.

Whenever laboratory results do not fit with the clinical scenario or patient, however, one final possibility should always be considered—laboratory error. Errors can be preanalytical (eg, patient misidentification), analytical, or postanalytical. In aggregate, the frequency of errors in laboratory results is 1 in 214 to 8,316.⁶ Given that even the more conservative estimates show an incidence higher than that of many of the rare diseases for which clinicians may be testing, laboratory error always deserves consideration.

COULD THIS BE PHEOCHROMOCYTOMA?

Pheochromocytoma is a neuroendocrine tumor most commonly arising from the chromaffin cells of the adrenal medulla. However, extra-adrenal pheochromocytoma, generally paraganglioma, accounts for 15% to 20% of these tumors. Although the condition is generally considered very rare, autopsy studies have demonstrated a prevalence of 0.05%, suggesting that many tumors are either missed or are not clinically significant.

The diagnosis is most often sought in hypertensive patients, a population in which pheochromocytoma has a prevalence of 0.1% to 0.6%.⁷

2. What is the most common presenting symptom of pheochromocytoma?

• Paroxysmal hypertension
• Sustained hypertension
• Nausea
• Cardiomyopathy
• Headache
• Hemorrhagic shock
• Psychological symptoms such as anxiety or panic

Although 50% to 60% of patients with pheochromocytoma have sustained hypertension, it may be absent in patients with primarily epinephrine-secreting tumors or large tumors that degrade catecholamines, leading to normal or low blood pressure.

Cardiomyopathy is a rare consequence of untreated pheochromocytoma, caused by the effects of excess circulating catecholamines over a long period of time.⁸ As seen in this patient, a prolonged QTc on ECG associated with elevated levels of norepinephrine and normetanephrine may be the only red flag.⁹

Pheochromocytoma is typically an extremely well-vascularized tumor, and rupture or hemorrhage is a rare but often fatal complication.

IMPORTANT FAMILY HISTORY

The classic “rule of 10s” suggests that 10% of pheochromocytomas are hereditary, but in fact the number may be higher. In a large cohort of patients with apparently sporadic pheochromocytoma, 25% were found to have germ-line mutations.¹⁰ This finding highlights the importance not only of obtaining a thorough family history, but also of genetic testing and counseling once the diagnosis has been made.

3. Which hereditary syndrome is not associated with pheochromocytoma?

• Von Hippel-Lindau syndrome
• Neurofibromatosis type 1
• Neurofibromatosis type 2
• Multiple endocrine neoplasia type 2
• Paraganglioma syndromes

Germ-line mutations in five genes related to three hereditary syndromes (von Hippel-Lindau, neurofibromatosis type 1, and multiple endocrine neoplasia type 2) and in two genes related to paraganglioma syndromes are known to be associated with pheochromocytoma.⁷

Von Hippel-Lindau syndrome

Von Hippel-Lindau syndrome affects 1 in 36,000 live births. It is caused by a mutation of the von Hippel-Lindau gene on chromosome 3, and 10% to 20% of patients with the syndrome have pheochromocytoma. Other associated problems include renal clear-cell carcinomas and cysts, central nervous system and retinal hemangioblastomas, pancreatic tumors and cysts, endolymphatic tumors, and epididymal cysts.

Neurofibromatosis type 1

Neurofibromatosis type 1 affects 1 in 2,500 to 3,000 individuals and is caused by a mutation of the neurofibromatosis type 1 gene on chromosome 17. The disease is diagnosed by the presence of café-au-lait macules, axillary or inguinal freckling (or both), dermal or plexiform neurofibromas, Lisch nodules, or osseous lesions, but the condition is associated with many other pathologic findings, including optic pathway gliomas, cardiovascular abnormalities, and, in up to 5.7% of patients, pheochromocytoma.¹¹

Neurofibromatosis type 2

Neurofibromatosis type 2 affects 1 in 25,000 live births and is caused by a mutation of the neurofibromatosis type 2 gene on chromosome 22. Patients often develop nervous system tumors, ophthalmologic pathology, and cutaneous lesions, but the condition is not associated with pheochromocytoma.¹²

Multiple endocrine neoplasia type 2

Multiple endocrine neoplasia type 2 affects 1 in 35,000 individuals and is caused by an activating mutation of the RET proto-oncogene on chromosome 21. The syndrome is most worrisome because of the 95% lifetime risk of medullary thyroid carcinoma in affected patients, but it is also associated with a 50% risk of pheochromocytoma and a 20% to 30% risk of primary hyperparathyroidism. Pheochromocytoma is the presenting clinical problem in 10% to 30% of patients.¹³

Paraganglioma syndromes

Paraganglioma syndromes are caused by mutations in the three genes encoding subunits of the succinate dehydrogenase enzyme. These mutations affect 1 in 30,000 to 100,000 individuals and incur a 70% lifetime risk of developing pheochromocytoma or paraganglioma.¹⁴

TESTING FOR AND MANAGING PHEOCHROMOCYTOMA

The consequences of untreated pheochromocytoma are potentially devastating and include progression to metastatic disease, hypertensive crises, cardiomyopathy, and adrenal hemorrhage. Nevertheless, the average patient goes 3 years before receiving the correct diagnosis.⁷ Consequently, heightened suspicion and tests with both high sensitivity and specificity are needed.

4. Which test for pheochromocytoma has the highest sensitivity?

• Plasma free metanephrines
• Plasma catecholamines
• Urine total metanephrines
• Urine fractionated metanephrines
• Urine catecholamines
• Urine vanillylmandelic acid

Some researchers have also examined plasma total metanephrines and found that any one of these three biochemical markers at a value two times greater than the upper limit of normal provides specificity of around 95%.¹⁶

Further laboratory tests in our patient

• Serum dopamine 70 pg/mL (reference range 0–20)
• Norepinephrine 2,018 pg/mL (80–520)
• Epinephrine 2,479 pg/mL (10–200)
• Free normetanephrine 12 pg/mL (< 0.9)
• Free metanephrine 17.8 pg/mL (< 0.5).

VALUE OF IMAGING STUDIES

Computed tomography has a sensitivity of up to 95% for detecting adrenal tumors and is able to detect tumors larger than 0.5 cm, but its specificity may be as low as 50%.¹⁷ Studies utilizing modern imaging equipment report a prevalence of adrenal incidentaloma of 4%, of which only 1.5% to 11% are pheochromocytoma.¹⁸ Thus, while the simultaneous occurrence of pheochromocytoma-like symptoms and an incidentaloma is not common, the potential for unnecessary surgery precludes diagnosis and treatment based on symptoms and imaging alone.

Magnetic resonance imaging has similar sensitivity and specificity but can better characterize the tumor’s blood supply and relationship to other structures.

Iodine 131 metaiodobenzylguanidine (MIBG) scanning is a physiologic study that uses a radiolabeled amine. Since it can identify pheochromocytoma regardless of location, MIBG scanning is typically used when pheochromocytoma is diagnosed by biochemical testing but CT and MRI fail to locate the lesion, or as a follow-up test in patients in whom recurrence or metastasis is suspected or documented.

The specificity of MIBG scanning is 95% to 100%, but the need to protect the thyroid from ablation and the potential need to repeat scans for up to 72 hours make it a poor choice for the initial evaluation.¹⁷

5. What is the next best step in our patient’s management?

• Treat her hypertension with a beta-blocker
• Begin a course of alpha-blockade
• Urgent surgery
• Observation

Because of the high concentration of circulating catecholamines and the instability of the tumor to physical manipulation, appropriate medical management before surgical resection is of paramount importance.

Beta-blockade can lead to malignant hypertension due to the unopposed alpha stimulation and must not be begun until alpha-blockade has been started. The standard of care is to give an alpha-blocker or calcium channel blocker 10 to 14 days before surgery. Typically, oral phenoxybenzamine (Dibenzyline) 10 mg twice daily is started and titrated upward daily by 10 to 20 mg until a target seated blood pressure of 120/80 mm Hg is obtained. Selective alpha-1 blockers such as prazosin (Minipress) and terazosin (Hytrin) have also been used and have the benefit of a preserved alpha-2 catecholamine reuptake mechanism.¹⁷

After several days, a beta-blocker may be added, particularly for patients with arrhythmias.⁷ In patients with refractory hypertension, metyrosine (Demser) can be useful.

During surgery, the patient’s hemodynamic stability and glucose levels can fluctuate rapidly from sudden releases of catecholamines during manipulation of the tumor, as well as from the sudden loss of catecholamines after ligation of draining vessels. Advances in medical care have reduced the perioperative death rate from 50% to less than 3%.^7,19

CASE CONCLUSION AND FOLLOW-UP

Two months after her initial presentation, the patient underwent open surgery and had the mass removed without complications. She reports that the “panic attacks” have ceased completely.

The recurrence rate of pheochromocytoma is 13% in patients with sporadic disease and 33% in patients with familial syndromes. The overall recurrence rate with long-term follow-up is 17%, half of recurrences being malignant disease. All patients should therefore be followed in the clinic annually for at least 10 years to identify and treat recurrences early,⁷ and many experts recommend lifelong follow-up, even for patients without hereditary syndromes.¹⁷

Nearly every diagnosis in the DSM-IV includes the caveat that medical causes of disease must be excluded before psychiatric labels can be applied. Although panic disorder and panic attack are far more common than pheochromocytoma, just as essential hypertension is far more common than pheochromocytoma, physicians need to remember that pheochromocytoma can cause symptoms common to both illnesses. Thus, while rare conditions are rare, atypical presentations of common conditions may deserve a second glance.

A 50-year-old woman presents to the emergency department because of repeated episodes of vomiting over the past 12 hours. She reports eight episodes of non-bloody, nonbilious emesis associated with palpitations and feelings of anxiety, but with no fever or diarrhea. She has not traveled recently and does not have any sick contacts.

She reports that she never had health problems until 6 months ago, when she began having panic attacks that woke her from sleep. The episodes first occurred once or twice per week, usually at night, and involved palpitations and feelings of anxiety that lasted 2 to 4 hours, but no other associated symptoms. After a month, the episodes began to occur more regularly during the day and were accompanied by a pounding headache that began in the back of her neck and extended up and over her head. Her primary care physician prescribed sertraline (Zoloft) and referred her to a neurologist to evaluate the headaches. The neurologic workup included brain magnetic resonance imaging and electroencephalography, both of which were normal.

After 8 weeks on sertraline, the episodes continued to increase in frequency and severity, and her physician switched her to paroxetine (Paxil) and added lorazepam (Ativan), which did not improve her symptoms. Over the past 2 months, during which time she has not been taking any medications, the episodes began to involve nausea and, more recently, vomiting, with episodes occurring as often as once or twice daily, and with intermittent symptom-free days. None of the prior episodes was accompanied by symptoms as severe as those she is currently experiencing.

She is otherwise healthy with no chronic diseases. Her surgical history includes resection of an angiolipoma from her right arm and dilation and curettage for endometrial polyps. She has no personal or family history of psychiatric illness.

PHYSICAL EXAMINATION

The patient is slender and tremulous but does not appear diaphoretic. Her blood pressure is 176/92 mm Hg, pulse 98, temperature 36.5°C (97.7°F), and respiratory rate 20 per minute. Oxygen saturation by pulse oximetry is 98% on room air. She has dry mucus membranes and orthostatic hypotension, but her physical examination is otherwise normal. Electrocardiography (ECG) shows a normal sinus rhythm with a prolonged QTc of 571 ms and peaked P and T waves.

LABORATORY VALUES

• Hemoglobin 15.6 g/dL (reference range 11.5–15.5)
• Hematocrit 47.2% (36.0–46.0)
• Platelet count 448 × 10⁹/L (150–400)
• White cell count 18.65 × 10⁹/L (3.70–11.00)
• Potassium 2.5 mmol/L (3.5–4.0)
• Chloride 97 mmol/L (98–110)
• Bicarbonate 21 mmol/L (23–32)
• Anion gap 20 mmol/L (0–15)
• Glucose 233 mg/dL (65–100).

Sodium, blood urea nitrogen, and creatinine levels are all within normal limits. Urinalysis suggests a urinary tract infection.

IS THIS A PANIC ATTACK?

1. Which of the following is not characteristic of a panic attack?

• Nausea and vomiting
• Onset during sleep
• Palpitations
• Chest pain or discomfort
• Headache
• Trembling or shaking

According to the Diagnostic and Statistical Manual of Mental Disorders (Fourth Edition) (DSM-IV), the diagnosis of panic attack requires the presence of intense fear or discomfort and four or more other symptoms that may come from any of six domains:

• Cardiovascular: palpitations, pounding heart, tachycardia, and chest pain or discomfort
• Autonomic: sweating, chills or hot flushes, and trembling or shaking
• Pulmonary: shortness of breath or a smothering sensation
• Neurologic: dizziness or light-headedness and paresthesias
• Gastrointestinal: choking and nausea or abdominal distress
• Psychological: compass derealization, depersonalization, and the fear of losing control or “going crazy.”¹

Two aspects of the patient’s history may be misinterpreted by those unfamiliar with the symptomatology of panic attack. First, although panic disorder carries an increased risk of many comorbidities, including migraine, headache is not typically associated with the panic attacks themselves.² Second, while not a part of the diagnostic criteria, sleep disturbances are common in patients with panic disorder, and 30% to 45% of patients with the disorder experience recurrent nocturnal panic attacks.³ Therefore, the correct answer is headache.

THE DIFFERENTIAL DIAGNOSIS

When considering a diagnosis of panic attack or panic disorder, the DSM-IV mandates that medical causes of the symptoms must be excluded. Common conditions causing a similar spectrum of symptoms include hyperthyroidism, caffeine and stimulant use or abuse, asthma, cardiac arrhythmias, alcohol withdrawal, and, more rarely, complex partial seizures and pheochromocytoma.^2,4 Many of these conditions can be ruled out by the history alone in a reliable patient.

Our patient’s electrocardiogram showed no evidence of ischemia or arrhythmias. Also, her recent negative neurologic workup makes seizure activity less likely.

Many of this patient’s laboratory abnormalities are easily explained by her repeated bouts of vomiting. Specifically, her elevated hemoglobin level and hematocrit are likely secondary to volume contraction, while hypochloremia is seen following losses of HCl with emesis. Typically, however, patients with vomiting have a hypochloremic metabolic alkalosis, and her low serum bicarbonate level is inconsistent with the history.

Three factors might be contributing to this patient’s hypokalemia. First, in a volume-depleted state, the cortical collecting tubules secrete potassium in exchange for increased sodium reabsorption in an attempt to correct volume status. Second, the alkalotic state caused by losses of acid with vomiting results in a transcellular shift of potassium ions into cells in exchange for hydrogen ions. Third, increased levels of epinephrine also cause a shift of potassium ions into cells.⁵ Potassium is not lost directly through nausea and vomiting.

A state of catecholamine excess, such as during a severe panic attack or in the presence of a catecholamine-secreting tumor, could explain many of her abnormalities. In addition to causing hypokalemia, epinephrine has a gluconeogenic effect, whereas norepinephrine inhibits insulin release, providing a potential explanation for hyperglycemia in a patient with no risk factors for diabetes. Finally, catecholamine excess contributes to lactic acidosis, which could help to explain the low serum bicarbonate level and the elevated anion gap, but unless we take arterial blood gas measurements, the patient’s acid-base status cannot be determined.

While panic attacks do stimulate the sympathetic nervous system, certain elements of her history raise the clinical suspicion for another process. First, the severity of the electrolyte abnormalities is suspicious. Second, a typical panic attack peaks at 10 minutes and begins to subside, whereas this woman’s symptoms have persisted for 12 hours. Finally, the clinical history, in particular the prominence of headaches associated with the symptoms, is inconsistent with classic panic attack. Consequently, an alternative diagnosis, such as pheochromocytoma, deserves more careful evaluation.

Whenever laboratory results do not fit with the clinical scenario or patient, however, one final possibility should always be considered—laboratory error. Errors can be preanalytical (eg, patient misidentification), analytical, or postanalytical. In aggregate, the frequency of errors in laboratory results is 1 in 214 to 8,316.⁶ Given that even the more conservative estimates show an incidence higher than that of many of the rare diseases for which clinicians may be testing, laboratory error always deserves consideration.

COULD THIS BE PHEOCHROMOCYTOMA?

Pheochromocytoma is a neuroendocrine tumor most commonly arising from the chromaffin cells of the adrenal medulla. However, extra-adrenal pheochromocytoma, generally paraganglioma, accounts for 15% to 20% of these tumors. Although the condition is generally considered very rare, autopsy studies have demonstrated a prevalence of 0.05%, suggesting that many tumors are either missed or are not clinically significant.

The diagnosis is most often sought in hypertensive patients, a population in which pheochromocytoma has a prevalence of 0.1% to 0.6%.⁷

2. What is the most common presenting symptom of pheochromocytoma?

• Paroxysmal hypertension
• Sustained hypertension
• Nausea
• Cardiomyopathy
• Headache
• Hemorrhagic shock
• Psychological symptoms such as anxiety or panic

Although 50% to 60% of patients with pheochromocytoma have sustained hypertension, it may be absent in patients with primarily epinephrine-secreting tumors or large tumors that degrade catecholamines, leading to normal or low blood pressure.

Cardiomyopathy is a rare consequence of untreated pheochromocytoma, caused by the effects of excess circulating catecholamines over a long period of time.⁸ As seen in this patient, a prolonged QTc on ECG associated with elevated levels of norepinephrine and normetanephrine may be the only red flag.⁹

Pheochromocytoma is typically an extremely well-vascularized tumor, and rupture or hemorrhage is a rare but often fatal complication.

IMPORTANT FAMILY HISTORY

The classic “rule of 10s” suggests that 10% of pheochromocytomas are hereditary, but in fact the number may be higher. In a large cohort of patients with apparently sporadic pheochromocytoma, 25% were found to have germ-line mutations.¹⁰ This finding highlights the importance not only of obtaining a thorough family history, but also of genetic testing and counseling once the diagnosis has been made.

3. Which hereditary syndrome is not associated with pheochromocytoma?

• Von Hippel-Lindau syndrome
• Neurofibromatosis type 1
• Neurofibromatosis type 2
• Multiple endocrine neoplasia type 2
• Paraganglioma syndromes

Germ-line mutations in five genes related to three hereditary syndromes (von Hippel-Lindau, neurofibromatosis type 1, and multiple endocrine neoplasia type 2) and in two genes related to paraganglioma syndromes are known to be associated with pheochromocytoma.⁷

Von Hippel-Lindau syndrome

Von Hippel-Lindau syndrome affects 1 in 36,000 live births. It is caused by a mutation of the von Hippel-Lindau gene on chromosome 3, and 10% to 20% of patients with the syndrome have pheochromocytoma. Other associated problems include renal clear-cell carcinomas and cysts, central nervous system and retinal hemangioblastomas, pancreatic tumors and cysts, endolymphatic tumors, and epididymal cysts.

Neurofibromatosis type 1

Neurofibromatosis type 1 affects 1 in 2,500 to 3,000 individuals and is caused by a mutation of the neurofibromatosis type 1 gene on chromosome 17. The disease is diagnosed by the presence of café-au-lait macules, axillary or inguinal freckling (or both), dermal or plexiform neurofibromas, Lisch nodules, or osseous lesions, but the condition is associated with many other pathologic findings, including optic pathway gliomas, cardiovascular abnormalities, and, in up to 5.7% of patients, pheochromocytoma.¹¹

Neurofibromatosis type 2

Neurofibromatosis type 2 affects 1 in 25,000 live births and is caused by a mutation of the neurofibromatosis type 2 gene on chromosome 22. Patients often develop nervous system tumors, ophthalmologic pathology, and cutaneous lesions, but the condition is not associated with pheochromocytoma.¹²

Multiple endocrine neoplasia type 2

Multiple endocrine neoplasia type 2 affects 1 in 35,000 individuals and is caused by an activating mutation of the RET proto-oncogene on chromosome 21. The syndrome is most worrisome because of the 95% lifetime risk of medullary thyroid carcinoma in affected patients, but it is also associated with a 50% risk of pheochromocytoma and a 20% to 30% risk of primary hyperparathyroidism. Pheochromocytoma is the presenting clinical problem in 10% to 30% of patients.¹³

Paraganglioma syndromes

Paraganglioma syndromes are caused by mutations in the three genes encoding subunits of the succinate dehydrogenase enzyme. These mutations affect 1 in 30,000 to 100,000 individuals and incur a 70% lifetime risk of developing pheochromocytoma or paraganglioma.¹⁴

TESTING FOR AND MANAGING PHEOCHROMOCYTOMA

The consequences of untreated pheochromocytoma are potentially devastating and include progression to metastatic disease, hypertensive crises, cardiomyopathy, and adrenal hemorrhage. Nevertheless, the average patient goes 3 years before receiving the correct diagnosis.⁷ Consequently, heightened suspicion and tests with both high sensitivity and specificity are needed.

4. Which test for pheochromocytoma has the highest sensitivity?

• Plasma free metanephrines
• Plasma catecholamines
• Urine total metanephrines
• Urine fractionated metanephrines
• Urine catecholamines
• Urine vanillylmandelic acid

Some researchers have also examined plasma total metanephrines and found that any one of these three biochemical markers at a value two times greater than the upper limit of normal provides specificity of around 95%.¹⁶

Further laboratory tests in our patient

• Serum dopamine 70 pg/mL (reference range 0–20)
• Norepinephrine 2,018 pg/mL (80–520)
• Epinephrine 2,479 pg/mL (10–200)
• Free normetanephrine 12 pg/mL (< 0.9)
• Free metanephrine 17.8 pg/mL (< 0.5).

VALUE OF IMAGING STUDIES

Computed tomography has a sensitivity of up to 95% for detecting adrenal tumors and is able to detect tumors larger than 0.5 cm, but its specificity may be as low as 50%.¹⁷ Studies utilizing modern imaging equipment report a prevalence of adrenal incidentaloma of 4%, of which only 1.5% to 11% are pheochromocytoma.¹⁸ Thus, while the simultaneous occurrence of pheochromocytoma-like symptoms and an incidentaloma is not common, the potential for unnecessary surgery precludes diagnosis and treatment based on symptoms and imaging alone.

Magnetic resonance imaging has similar sensitivity and specificity but can better characterize the tumor’s blood supply and relationship to other structures.

Iodine 131 metaiodobenzylguanidine (MIBG) scanning is a physiologic study that uses a radiolabeled amine. Since it can identify pheochromocytoma regardless of location, MIBG scanning is typically used when pheochromocytoma is diagnosed by biochemical testing but CT and MRI fail to locate the lesion, or as a follow-up test in patients in whom recurrence or metastasis is suspected or documented.

The specificity of MIBG scanning is 95% to 100%, but the need to protect the thyroid from ablation and the potential need to repeat scans for up to 72 hours make it a poor choice for the initial evaluation.¹⁷

5. What is the next best step in our patient’s management?

• Treat her hypertension with a beta-blocker
• Begin a course of alpha-blockade
• Urgent surgery
• Observation

Because of the high concentration of circulating catecholamines and the instability of the tumor to physical manipulation, appropriate medical management before surgical resection is of paramount importance.

Beta-blockade can lead to malignant hypertension due to the unopposed alpha stimulation and must not be begun until alpha-blockade has been started. The standard of care is to give an alpha-blocker or calcium channel blocker 10 to 14 days before surgery. Typically, oral phenoxybenzamine (Dibenzyline) 10 mg twice daily is started and titrated upward daily by 10 to 20 mg until a target seated blood pressure of 120/80 mm Hg is obtained. Selective alpha-1 blockers such as prazosin (Minipress) and terazosin (Hytrin) have also been used and have the benefit of a preserved alpha-2 catecholamine reuptake mechanism.¹⁷

After several days, a beta-blocker may be added, particularly for patients with arrhythmias.⁷ In patients with refractory hypertension, metyrosine (Demser) can be useful.

During surgery, the patient’s hemodynamic stability and glucose levels can fluctuate rapidly from sudden releases of catecholamines during manipulation of the tumor, as well as from the sudden loss of catecholamines after ligation of draining vessels. Advances in medical care have reduced the perioperative death rate from 50% to less than 3%.^7,19

CASE CONCLUSION AND FOLLOW-UP

Two months after her initial presentation, the patient underwent open surgery and had the mass removed without complications. She reports that the “panic attacks” have ceased completely.

The recurrence rate of pheochromocytoma is 13% in patients with sporadic disease and 33% in patients with familial syndromes. The overall recurrence rate with long-term follow-up is 17%, half of recurrences being malignant disease. All patients should therefore be followed in the clinic annually for at least 10 years to identify and treat recurrences early,⁷ and many experts recommend lifelong follow-up, even for patients without hereditary syndromes.¹⁷

Nearly every diagnosis in the DSM-IV includes the caveat that medical causes of disease must be excluded before psychiatric labels can be applied. Although panic disorder and panic attack are far more common than pheochromocytoma, just as essential hypertension is far more common than pheochromocytoma, physicians need to remember that pheochromocytoma can cause symptoms common to both illnesses. Thus, while rare conditions are rare, atypical presentations of common conditions may deserve a second glance.

A 50-year-old woman presents to the emergency department because of repeated episodes of vomiting over the past 12 hours. She reports eight episodes of non-bloody, nonbilious emesis associated with palpitations and feelings of anxiety, but with no fever or diarrhea. She has not traveled recently and does not have any sick contacts.

She reports that she never had health problems until 6 months ago, when she began having panic attacks that woke her from sleep. The episodes first occurred once or twice per week, usually at night, and involved palpitations and feelings of anxiety that lasted 2 to 4 hours, but no other associated symptoms. After a month, the episodes began to occur more regularly during the day and were accompanied by a pounding headache that began in the back of her neck and extended up and over her head. Her primary care physician prescribed sertraline (Zoloft) and referred her to a neurologist to evaluate the headaches. The neurologic workup included brain magnetic resonance imaging and electroencephalography, both of which were normal.

After 8 weeks on sertraline, the episodes continued to increase in frequency and severity, and her physician switched her to paroxetine (Paxil) and added lorazepam (Ativan), which did not improve her symptoms. Over the past 2 months, during which time she has not been taking any medications, the episodes began to involve nausea and, more recently, vomiting, with episodes occurring as often as once or twice daily, and with intermittent symptom-free days. None of the prior episodes was accompanied by symptoms as severe as those she is currently experiencing.

She is otherwise healthy with no chronic diseases. Her surgical history includes resection of an angiolipoma from her right arm and dilation and curettage for endometrial polyps. She has no personal or family history of psychiatric illness.

PHYSICAL EXAMINATION

The patient is slender and tremulous but does not appear diaphoretic. Her blood pressure is 176/92 mm Hg, pulse 98, temperature 36.5°C (97.7°F), and respiratory rate 20 per minute. Oxygen saturation by pulse oximetry is 98% on room air. She has dry mucus membranes and orthostatic hypotension, but her physical examination is otherwise normal. Electrocardiography (ECG) shows a normal sinus rhythm with a prolonged QTc of 571 ms and peaked P and T waves.

LABORATORY VALUES

• Hemoglobin 15.6 g/dL (reference range 11.5–15.5)
• Hematocrit 47.2% (36.0–46.0)
• Platelet count 448 × 10⁹/L (150–400)
• White cell count 18.65 × 10⁹/L (3.70–11.00)
• Potassium 2.5 mmol/L (3.5–4.0)
• Chloride 97 mmol/L (98–110)
• Bicarbonate 21 mmol/L (23–32)
• Anion gap 20 mmol/L (0–15)
• Glucose 233 mg/dL (65–100).

Sodium, blood urea nitrogen, and creatinine levels are all within normal limits. Urinalysis suggests a urinary tract infection.

IS THIS A PANIC ATTACK?

1. Which of the following is not characteristic of a panic attack?

• Nausea and vomiting
• Onset during sleep
• Palpitations
• Chest pain or discomfort
• Headache
• Trembling or shaking

According to the Diagnostic and Statistical Manual of Mental Disorders (Fourth Edition) (DSM-IV), the diagnosis of panic attack requires the presence of intense fear or discomfort and four or more other symptoms that may come from any of six domains:

• Cardiovascular: palpitations, pounding heart, tachycardia, and chest pain or discomfort
• Autonomic: sweating, chills or hot flushes, and trembling or shaking
• Pulmonary: shortness of breath or a smothering sensation
• Neurologic: dizziness or light-headedness and paresthesias
• Gastrointestinal: choking and nausea or abdominal distress
• Psychological: compass derealization, depersonalization, and the fear of losing control or “going crazy.”¹

Two aspects of the patient’s history may be misinterpreted by those unfamiliar with the symptomatology of panic attack. First, although panic disorder carries an increased risk of many comorbidities, including migraine, headache is not typically associated with the panic attacks themselves.² Second, while not a part of the diagnostic criteria, sleep disturbances are common in patients with panic disorder, and 30% to 45% of patients with the disorder experience recurrent nocturnal panic attacks.³ Therefore, the correct answer is headache.

THE DIFFERENTIAL DIAGNOSIS

When considering a diagnosis of panic attack or panic disorder, the DSM-IV mandates that medical causes of the symptoms must be excluded. Common conditions causing a similar spectrum of symptoms include hyperthyroidism, caffeine and stimulant use or abuse, asthma, cardiac arrhythmias, alcohol withdrawal, and, more rarely, complex partial seizures and pheochromocytoma.^2,4 Many of these conditions can be ruled out by the history alone in a reliable patient.

Our patient’s electrocardiogram showed no evidence of ischemia or arrhythmias. Also, her recent negative neurologic workup makes seizure activity less likely.

Many of this patient’s laboratory abnormalities are easily explained by her repeated bouts of vomiting. Specifically, her elevated hemoglobin level and hematocrit are likely secondary to volume contraction, while hypochloremia is seen following losses of HCl with emesis. Typically, however, patients with vomiting have a hypochloremic metabolic alkalosis, and her low serum bicarbonate level is inconsistent with the history.

Three factors might be contributing to this patient’s hypokalemia. First, in a volume-depleted state, the cortical collecting tubules secrete potassium in exchange for increased sodium reabsorption in an attempt to correct volume status. Second, the alkalotic state caused by losses of acid with vomiting results in a transcellular shift of potassium ions into cells in exchange for hydrogen ions. Third, increased levels of epinephrine also cause a shift of potassium ions into cells.⁵ Potassium is not lost directly through nausea and vomiting.

A state of catecholamine excess, such as during a severe panic attack or in the presence of a catecholamine-secreting tumor, could explain many of her abnormalities. In addition to causing hypokalemia, epinephrine has a gluconeogenic effect, whereas norepinephrine inhibits insulin release, providing a potential explanation for hyperglycemia in a patient with no risk factors for diabetes. Finally, catecholamine excess contributes to lactic acidosis, which could help to explain the low serum bicarbonate level and the elevated anion gap, but unless we take arterial blood gas measurements, the patient’s acid-base status cannot be determined.

While panic attacks do stimulate the sympathetic nervous system, certain elements of her history raise the clinical suspicion for another process. First, the severity of the electrolyte abnormalities is suspicious. Second, a typical panic attack peaks at 10 minutes and begins to subside, whereas this woman’s symptoms have persisted for 12 hours. Finally, the clinical history, in particular the prominence of headaches associated with the symptoms, is inconsistent with classic panic attack. Consequently, an alternative diagnosis, such as pheochromocytoma, deserves more careful evaluation.

Whenever laboratory results do not fit with the clinical scenario or patient, however, one final possibility should always be considered—laboratory error. Errors can be preanalytical (eg, patient misidentification), analytical, or postanalytical. In aggregate, the frequency of errors in laboratory results is 1 in 214 to 8,316.⁶ Given that even the more conservative estimates show an incidence higher than that of many of the rare diseases for which clinicians may be testing, laboratory error always deserves consideration.

COULD THIS BE PHEOCHROMOCYTOMA?

Pheochromocytoma is a neuroendocrine tumor most commonly arising from the chromaffin cells of the adrenal medulla. However, extra-adrenal pheochromocytoma, generally paraganglioma, accounts for 15% to 20% of these tumors. Although the condition is generally considered very rare, autopsy studies have demonstrated a prevalence of 0.05%, suggesting that many tumors are either missed or are not clinically significant.

The diagnosis is most often sought in hypertensive patients, a population in which pheochromocytoma has a prevalence of 0.1% to 0.6%.⁷

2. What is the most common presenting symptom of pheochromocytoma?

• Paroxysmal hypertension
• Sustained hypertension
• Nausea
• Cardiomyopathy
• Headache
• Hemorrhagic shock
• Psychological symptoms such as anxiety or panic

Although 50% to 60% of patients with pheochromocytoma have sustained hypertension, it may be absent in patients with primarily epinephrine-secreting tumors or large tumors that degrade catecholamines, leading to normal or low blood pressure.

Cardiomyopathy is a rare consequence of untreated pheochromocytoma, caused by the effects of excess circulating catecholamines over a long period of time.⁸ As seen in this patient, a prolonged QTc on ECG associated with elevated levels of norepinephrine and normetanephrine may be the only red flag.⁹

Pheochromocytoma is typically an extremely well-vascularized tumor, and rupture or hemorrhage is a rare but often fatal complication.

IMPORTANT FAMILY HISTORY

The classic “rule of 10s” suggests that 10% of pheochromocytomas are hereditary, but in fact the number may be higher. In a large cohort of patients with apparently sporadic pheochromocytoma, 25% were found to have germ-line mutations.¹⁰ This finding highlights the importance not only of obtaining a thorough family history, but also of genetic testing and counseling once the diagnosis has been made.

3. Which hereditary syndrome is not associated with pheochromocytoma?

• Von Hippel-Lindau syndrome
• Neurofibromatosis type 1
• Neurofibromatosis type 2
• Multiple endocrine neoplasia type 2
• Paraganglioma syndromes

Germ-line mutations in five genes related to three hereditary syndromes (von Hippel-Lindau, neurofibromatosis type 1, and multiple endocrine neoplasia type 2) and in two genes related to paraganglioma syndromes are known to be associated with pheochromocytoma.⁷

Von Hippel-Lindau syndrome

Von Hippel-Lindau syndrome affects 1 in 36,000 live births. It is caused by a mutation of the von Hippel-Lindau gene on chromosome 3, and 10% to 20% of patients with the syndrome have pheochromocytoma. Other associated problems include renal clear-cell carcinomas and cysts, central nervous system and retinal hemangioblastomas, pancreatic tumors and cysts, endolymphatic tumors, and epididymal cysts.

Neurofibromatosis type 1

Neurofibromatosis type 1 affects 1 in 2,500 to 3,000 individuals and is caused by a mutation of the neurofibromatosis type 1 gene on chromosome 17. The disease is diagnosed by the presence of café-au-lait macules, axillary or inguinal freckling (or both), dermal or plexiform neurofibromas, Lisch nodules, or osseous lesions, but the condition is associated with many other pathologic findings, including optic pathway gliomas, cardiovascular abnormalities, and, in up to 5.7% of patients, pheochromocytoma.¹¹

Neurofibromatosis type 2

Neurofibromatosis type 2 affects 1 in 25,000 live births and is caused by a mutation of the neurofibromatosis type 2 gene on chromosome 22. Patients often develop nervous system tumors, ophthalmologic pathology, and cutaneous lesions, but the condition is not associated with pheochromocytoma.¹²

Multiple endocrine neoplasia type 2

Multiple endocrine neoplasia type 2 affects 1 in 35,000 individuals and is caused by an activating mutation of the RET proto-oncogene on chromosome 21. The syndrome is most worrisome because of the 95% lifetime risk of medullary thyroid carcinoma in affected patients, but it is also associated with a 50% risk of pheochromocytoma and a 20% to 30% risk of primary hyperparathyroidism. Pheochromocytoma is the presenting clinical problem in 10% to 30% of patients.¹³

Paraganglioma syndromes

Paraganglioma syndromes are caused by mutations in the three genes encoding subunits of the succinate dehydrogenase enzyme. These mutations affect 1 in 30,000 to 100,000 individuals and incur a 70% lifetime risk of developing pheochromocytoma or paraganglioma.¹⁴

TESTING FOR AND MANAGING PHEOCHROMOCYTOMA

The consequences of untreated pheochromocytoma are potentially devastating and include progression to metastatic disease, hypertensive crises, cardiomyopathy, and adrenal hemorrhage. Nevertheless, the average patient goes 3 years before receiving the correct diagnosis.⁷ Consequently, heightened suspicion and tests with both high sensitivity and specificity are needed.

4. Which test for pheochromocytoma has the highest sensitivity?

• Plasma free metanephrines
• Plasma catecholamines
• Urine total metanephrines
• Urine fractionated metanephrines
• Urine catecholamines
• Urine vanillylmandelic acid

Some researchers have also examined plasma total metanephrines and found that any one of these three biochemical markers at a value two times greater than the upper limit of normal provides specificity of around 95%.¹⁶

Further laboratory tests in our patient

• Serum dopamine 70 pg/mL (reference range 0–20)
• Norepinephrine 2,018 pg/mL (80–520)
• Epinephrine 2,479 pg/mL (10–200)
• Free normetanephrine 12 pg/mL (< 0.9)
• Free metanephrine 17.8 pg/mL (< 0.5).

VALUE OF IMAGING STUDIES

Computed tomography has a sensitivity of up to 95% for detecting adrenal tumors and is able to detect tumors larger than 0.5 cm, but its specificity may be as low as 50%.¹⁷ Studies utilizing modern imaging equipment report a prevalence of adrenal incidentaloma of 4%, of which only 1.5% to 11% are pheochromocytoma.¹⁸ Thus, while the simultaneous occurrence of pheochromocytoma-like symptoms and an incidentaloma is not common, the potential for unnecessary surgery precludes diagnosis and treatment based on symptoms and imaging alone.

Magnetic resonance imaging has similar sensitivity and specificity but can better characterize the tumor’s blood supply and relationship to other structures.

Iodine 131 metaiodobenzylguanidine (MIBG) scanning is a physiologic study that uses a radiolabeled amine. Since it can identify pheochromocytoma regardless of location, MIBG scanning is typically used when pheochromocytoma is diagnosed by biochemical testing but CT and MRI fail to locate the lesion, or as a follow-up test in patients in whom recurrence or metastasis is suspected or documented.

The specificity of MIBG scanning is 95% to 100%, but the need to protect the thyroid from ablation and the potential need to repeat scans for up to 72 hours make it a poor choice for the initial evaluation.¹⁷

5. What is the next best step in our patient’s management?

• Treat her hypertension with a beta-blocker
• Begin a course of alpha-blockade
• Urgent surgery
• Observation

Because of the high concentration of circulating catecholamines and the instability of the tumor to physical manipulation, appropriate medical management before surgical resection is of paramount importance.

Beta-blockade can lead to malignant hypertension due to the unopposed alpha stimulation and must not be begun until alpha-blockade has been started. The standard of care is to give an alpha-blocker or calcium channel blocker 10 to 14 days before surgery. Typically, oral phenoxybenzamine (Dibenzyline) 10 mg twice daily is started and titrated upward daily by 10 to 20 mg until a target seated blood pressure of 120/80 mm Hg is obtained. Selective alpha-1 blockers such as prazosin (Minipress) and terazosin (Hytrin) have also been used and have the benefit of a preserved alpha-2 catecholamine reuptake mechanism.¹⁷

After several days, a beta-blocker may be added, particularly for patients with arrhythmias.⁷ In patients with refractory hypertension, metyrosine (Demser) can be useful.

During surgery, the patient’s hemodynamic stability and glucose levels can fluctuate rapidly from sudden releases of catecholamines during manipulation of the tumor, as well as from the sudden loss of catecholamines after ligation of draining vessels. Advances in medical care have reduced the perioperative death rate from 50% to less than 3%.^7,19

CASE CONCLUSION AND FOLLOW-UP

Two months after her initial presentation, the patient underwent open surgery and had the mass removed without complications. She reports that the “panic attacks” have ceased completely.

The recurrence rate of pheochromocytoma is 13% in patients with sporadic disease and 33% in patients with familial syndromes. The overall recurrence rate with long-term follow-up is 17%, half of recurrences being malignant disease. All patients should therefore be followed in the clinic annually for at least 10 years to identify and treat recurrences early,⁷ and many experts recommend lifelong follow-up, even for patients without hereditary syndromes.¹⁷

Nearly every diagnosis in the DSM-IV includes the caveat that medical causes of disease must be excluded before psychiatric labels can be applied. Although panic disorder and panic attack are far more common than pheochromocytoma, just as essential hypertension is far more common than pheochromocytoma, physicians need to remember that pheochromocytoma can cause symptoms common to both illnesses. Thus, while rare conditions are rare, atypical presentations of common conditions may deserve a second glance.

Q: What is the most likely diagnosis?

• Leukemia cutis
• Drug reaction
• Sweet syndrome
• Erythema multiforme
• Urticaria

A: The correct answer is leukemia cutis, defined as a cutaneous infiltration by neoplastic leukocytes.¹ When the leukocytes are primarily granulocytic precursors, the terms myeloid sarcoma, granulocytic sarcoma, chloroma, and primary extramedullary leukemia have been used.² The term monoblastic sarcoma has been used when the cutaneous infiltrate is composed of neoplastic monocytic precursors.²

Leukemia cutis most commonly manifests as erythematous papules and nodules, single or multiple, of varying sizes, and afflicting one or more body sites; it typically is asymptomatic.³ It occurs in 10% to 15% of patients with acute myeloid leukemia⁴ and is itself a poor prognostic sign.⁵ The cutaneous changes may pre-date the hematologic manifestations and may even herald a relapse.⁶

In patients presenting with extramedullary leukemia and no bone marrow or blood involvement, the importance of preemptive chemotherapy for acute myelogenous leukemia has recently been emphasized.⁷

CASE CONTINUED

The patient undergoes further testing with bone marrow aspiration and trephination, which are diagnostic of acute myeloid leukemia with myelodysplastic changes: the studies reveal a clear excess of myeloblasts (accounting for 40% to 50% of nucleated cells) and clearly dysplastic erythropoiesis and myelopoiesis. Bone marrow cytogenetic analysis reveals a complex abnormal female karyotype with multiple numerical and structural abnormalities, in particular deletion of the long arm of chromosome 5, suggestive of a poor prognosis.

Skin biopsy reveals a normal epidermis but dermal perivascular involvement with a reactive T-lymphocyte infiltrate associated with immature myeloid elements, characterized by positive staining to myeloperoxidase, in keeping with leukemia cutis. It should be noted that histopathologic confirmation of leukemia cutis can be challenging, as the condition can adopt a variety of patterns, and clinicopathologic correlation is often warranted.

The patient is treated with a cycle of cytarabine-based chemotherapy, and her skin eruption transiently improves. However, her clinical condition subsequently deteriorates; she has a relapse of leukemia, with the rash returning more florid and angry-looking than previously. She is subsequently managed palliatively and passes away 3 weeks later.

THE OTHER DIAGNOSTIC CHOICES

Sweet syndrome or acute febrile neutrophilic dermatosis is often seen in association with hematologic malignancies, but the lesions are typically tender, and histopathology reveals an intense dermal neutrophilic infiltrate.⁶

Erythema multiforme is associated with malignancy, but its characteristic concentric “target” lesions are typically acral and symmetrical in their distribution; their histopathology is inflammatory.⁸

The patient had not been on any regular medications and her rash could not have been medication-induced.

Urticaria presents with pruritic evanescent wheals, which rarely last more than 12 hours.⁹ Our patient had a fixed and entirely asymptomatic rash, which in addition did not have the histopathologic features of urticaria—namely, dermal edema involved with an infiltrate made of lymphocytes and eosinophils.⁹

Q: What is the most likely diagnosis?

• Leukemia cutis
• Drug reaction
• Sweet syndrome
• Erythema multiforme
• Urticaria

A: The correct answer is leukemia cutis, defined as a cutaneous infiltration by neoplastic leukocytes.¹ When the leukocytes are primarily granulocytic precursors, the terms myeloid sarcoma, granulocytic sarcoma, chloroma, and primary extramedullary leukemia have been used.² The term monoblastic sarcoma has been used when the cutaneous infiltrate is composed of neoplastic monocytic precursors.²

Leukemia cutis most commonly manifests as erythematous papules and nodules, single or multiple, of varying sizes, and afflicting one or more body sites; it typically is asymptomatic.³ It occurs in 10% to 15% of patients with acute myeloid leukemia⁴ and is itself a poor prognostic sign.⁵ The cutaneous changes may pre-date the hematologic manifestations and may even herald a relapse.⁶

In patients presenting with extramedullary leukemia and no bone marrow or blood involvement, the importance of preemptive chemotherapy for acute myelogenous leukemia has recently been emphasized.⁷

CASE CONTINUED

The patient undergoes further testing with bone marrow aspiration and trephination, which are diagnostic of acute myeloid leukemia with myelodysplastic changes: the studies reveal a clear excess of myeloblasts (accounting for 40% to 50% of nucleated cells) and clearly dysplastic erythropoiesis and myelopoiesis. Bone marrow cytogenetic analysis reveals a complex abnormal female karyotype with multiple numerical and structural abnormalities, in particular deletion of the long arm of chromosome 5, suggestive of a poor prognosis.

Skin biopsy reveals a normal epidermis but dermal perivascular involvement with a reactive T-lymphocyte infiltrate associated with immature myeloid elements, characterized by positive staining to myeloperoxidase, in keeping with leukemia cutis. It should be noted that histopathologic confirmation of leukemia cutis can be challenging, as the condition can adopt a variety of patterns, and clinicopathologic correlation is often warranted.

The patient is treated with a cycle of cytarabine-based chemotherapy, and her skin eruption transiently improves. However, her clinical condition subsequently deteriorates; she has a relapse of leukemia, with the rash returning more florid and angry-looking than previously. She is subsequently managed palliatively and passes away 3 weeks later.

THE OTHER DIAGNOSTIC CHOICES

Sweet syndrome or acute febrile neutrophilic dermatosis is often seen in association with hematologic malignancies, but the lesions are typically tender, and histopathology reveals an intense dermal neutrophilic infiltrate.⁶

Erythema multiforme is associated with malignancy, but its characteristic concentric “target” lesions are typically acral and symmetrical in their distribution; their histopathology is inflammatory.⁸

The patient had not been on any regular medications and her rash could not have been medication-induced.

Urticaria presents with pruritic evanescent wheals, which rarely last more than 12 hours.⁹ Our patient had a fixed and entirely asymptomatic rash, which in addition did not have the histopathologic features of urticaria—namely, dermal edema involved with an infiltrate made of lymphocytes and eosinophils.⁹

Q: What is the most likely diagnosis?

• Leukemia cutis
• Drug reaction
• Sweet syndrome
• Erythema multiforme
• Urticaria

A: The correct answer is leukemia cutis, defined as a cutaneous infiltration by neoplastic leukocytes.¹ When the leukocytes are primarily granulocytic precursors, the terms myeloid sarcoma, granulocytic sarcoma, chloroma, and primary extramedullary leukemia have been used.² The term monoblastic sarcoma has been used when the cutaneous infiltrate is composed of neoplastic monocytic precursors.²

Leukemia cutis most commonly manifests as erythematous papules and nodules, single or multiple, of varying sizes, and afflicting one or more body sites; it typically is asymptomatic.³ It occurs in 10% to 15% of patients with acute myeloid leukemia⁴ and is itself a poor prognostic sign.⁵ The cutaneous changes may pre-date the hematologic manifestations and may even herald a relapse.⁶

In patients presenting with extramedullary leukemia and no bone marrow or blood involvement, the importance of preemptive chemotherapy for acute myelogenous leukemia has recently been emphasized.⁷

CASE CONTINUED

The patient undergoes further testing with bone marrow aspiration and trephination, which are diagnostic of acute myeloid leukemia with myelodysplastic changes: the studies reveal a clear excess of myeloblasts (accounting for 40% to 50% of nucleated cells) and clearly dysplastic erythropoiesis and myelopoiesis. Bone marrow cytogenetic analysis reveals a complex abnormal female karyotype with multiple numerical and structural abnormalities, in particular deletion of the long arm of chromosome 5, suggestive of a poor prognosis.

Skin biopsy reveals a normal epidermis but dermal perivascular involvement with a reactive T-lymphocyte infiltrate associated with immature myeloid elements, characterized by positive staining to myeloperoxidase, in keeping with leukemia cutis. It should be noted that histopathologic confirmation of leukemia cutis can be challenging, as the condition can adopt a variety of patterns, and clinicopathologic correlation is often warranted.

The patient is treated with a cycle of cytarabine-based chemotherapy, and her skin eruption transiently improves. However, her clinical condition subsequently deteriorates; she has a relapse of leukemia, with the rash returning more florid and angry-looking than previously. She is subsequently managed palliatively and passes away 3 weeks later.

THE OTHER DIAGNOSTIC CHOICES

Sweet syndrome or acute febrile neutrophilic dermatosis is often seen in association with hematologic malignancies, but the lesions are typically tender, and histopathology reveals an intense dermal neutrophilic infiltrate.⁶

Erythema multiforme is associated with malignancy, but its characteristic concentric “target” lesions are typically acral and symmetrical in their distribution; their histopathology is inflammatory.⁸

The patient had not been on any regular medications and her rash could not have been medication-induced.

Urticaria presents with pruritic evanescent wheals, which rarely last more than 12 hours.⁹ Our patient had a fixed and entirely asymptomatic rash, which in addition did not have the histopathologic features of urticaria—namely, dermal edema involved with an infiltrate made of lymphocytes and eosinophils.⁹