More Clinical Disorders Are Linked to APS

DESTIN, FLA. — The clinical spectrum of antiphospholipid syndrome has broadened substantially to include a range of clinical manifestations, including heart valve disease, livedo reticularis, thrombocytopenia, stroke, migraine, seizures, and cognitive dysfunction.

The potential cardiovascular and neurologic complications of antiphospholipid syndrome are especially concerning, said Dr. Graham Hughes, who along with his colleagues first described the prothrombotic condition in 1983.

“The clinical map is still being charted, and a number of recent clinical observations have added to our understanding of it,” said Dr. Hughes, professor of medicine at St. Thomas Hospital in London and director of the London Lupus Centre at London Bridge Hospital.

Antiphospholipid syndrome (APS), also called Hughes syndrome, is generally recognized as an autoimmune condition characterized by arterial and venous vascular thromboses and pregnancy complications in the presence of antiphospholipid (aPL) antibodies.

Dr. Hughes discussed several recent studies that argue for greater awareness, screening, and diagnosis of APS in primary care.

“We are seeing that APS is a major cause of heart attack, stroke, miscarriage, cognitive dysfunction, movement disorders, epilepsy and other conditions. There is a huge cost to delaying the diagnosis.”

If the syndrome is suspected, he said, “it's easy to diagnose and highly treatable,” primarily with aspirin, heparin, or warfarin.

A study of 344 acute coronary syndrome patients showed that 40% of the patients were aPL positive in one or more tests (Am. J. Clin. Pathol. 2009;132:613-20).

Additionally, in a report published in Lancet Neurology, the study population of women younger than 50 years of age who had experienced myocardial infarction or stroke had five times the risk of MI and a 40-fold increase in the risk of stroke, compared with matched healthy controls (Lancet Neurol. 2009;8:998-1005).

“The brain appears to be particularly susceptible to the syndrome,” Dr. Hughes stated, noting that, in addition to stroke—which is now “internationally recognized as an important manifestation” of APS—other neurologic presentations include migraine, memory loss, movement disorders, atypical multiple sclerosis, and epilepsy.

Importantly, new data suggest that a clinical link between migraine, stroke, and APS “is a very real possibility,” Dr. Hughes reported.

“Migraine and stroke are both prominent features of APS, and our own clinical experience with lupus patients at St. Thomas Hospital has been that many younger individuals with APS who have had a stroke—especially females—report a history of migraines prior to the stroke and a dramatic improvement in migraine during anticoagulation treatment,” he said. “Although the evidence is largely anecdotal, it may be that APS is a significant missing link between migraine and stroke.” In fact, he added, “it is now our practice in patients with [APS] and severe migraine attacks to use low-molecular-weight heparin to treat migraine and, potentially, avert stroke.”

In addition to rheumatologists, cardiologists, and neurologists, “more and more specialists are seeing patients with APS, including orthopedists and ear, nose, and throat surgeons,” Dr. Hughes noted, explaining that prospective cohort and case studies have demonstrated an association between aPL antibodies and avascular necrosis, often presenting as nontraumatic metatarsal, rib, and other fractures in individuals with no history of osteoporosis; in addition, middle-ear balance problems such as dizziness, acute vertigo, and tinnitus are all seen in APS.

A study of endothelial assessment in patients with APS suggests that preclinical atherosclerosis may be an important feature of APS.

Specifically, the results of high-resolution ultrasound studies showed significantly reduced endothelium-dependent, flow-mediated dilatation and increased carotid intima media thickness among 90 APS patients, compared with 90 age-, sex-, and cardiovascular risk factor–matched aPL-negative controls (Arthritis Rheum. 2006;54:557-8).

“We still don't know the exact mechanism for thrombosis. Studies have shown that aPL antibodies alter platelets, clotting proteins, and the blood vessel lining, but how each of these mechanisms contributes to APS is uncertain,” he said. “We also don't understand why patients get better when we anticoagulate.”

Disclosures: Dr. Hughes had no financial disclosures relative to his presentation.

DESTIN, FLA. — The clinical spectrum of antiphospholipid syndrome has broadened substantially to include a range of clinical manifestations, including heart valve disease, livedo reticularis, thrombocytopenia, stroke, migraine, seizures, and cognitive dysfunction.

The potential cardiovascular and neurologic complications of antiphospholipid syndrome are especially concerning, said Dr. Graham Hughes, who along with his colleagues first described the prothrombotic condition in 1983.

“The clinical map is still being charted, and a number of recent clinical observations have added to our understanding of it,” said Dr. Hughes, professor of medicine at St. Thomas Hospital in London and director of the London Lupus Centre at London Bridge Hospital.

Antiphospholipid syndrome (APS), also called Hughes syndrome, is generally recognized as an autoimmune condition characterized by arterial and venous vascular thromboses and pregnancy complications in the presence of antiphospholipid (aPL) antibodies.

Dr. Hughes discussed several recent studies that argue for greater awareness, screening, and diagnosis of APS in primary care.

“We are seeing that APS is a major cause of heart attack, stroke, miscarriage, cognitive dysfunction, movement disorders, epilepsy and other conditions. There is a huge cost to delaying the diagnosis.”

If the syndrome is suspected, he said, “it's easy to diagnose and highly treatable,” primarily with aspirin, heparin, or warfarin.

A study of 344 acute coronary syndrome patients showed that 40% of the patients were aPL positive in one or more tests (Am. J. Clin. Pathol. 2009;132:613-20).

Additionally, in a report published in Lancet Neurology, the study population of women younger than 50 years of age who had experienced myocardial infarction or stroke had five times the risk of MI and a 40-fold increase in the risk of stroke, compared with matched healthy controls (Lancet Neurol. 2009;8:998-1005).

“The brain appears to be particularly susceptible to the syndrome,” Dr. Hughes stated, noting that, in addition to stroke—which is now “internationally recognized as an important manifestation” of APS—other neurologic presentations include migraine, memory loss, movement disorders, atypical multiple sclerosis, and epilepsy.

Importantly, new data suggest that a clinical link between migraine, stroke, and APS “is a very real possibility,” Dr. Hughes reported.

“Migraine and stroke are both prominent features of APS, and our own clinical experience with lupus patients at St. Thomas Hospital has been that many younger individuals with APS who have had a stroke—especially females—report a history of migraines prior to the stroke and a dramatic improvement in migraine during anticoagulation treatment,” he said. “Although the evidence is largely anecdotal, it may be that APS is a significant missing link between migraine and stroke.” In fact, he added, “it is now our practice in patients with [APS] and severe migraine attacks to use low-molecular-weight heparin to treat migraine and, potentially, avert stroke.”

In addition to rheumatologists, cardiologists, and neurologists, “more and more specialists are seeing patients with APS, including orthopedists and ear, nose, and throat surgeons,” Dr. Hughes noted, explaining that prospective cohort and case studies have demonstrated an association between aPL antibodies and avascular necrosis, often presenting as nontraumatic metatarsal, rib, and other fractures in individuals with no history of osteoporosis; in addition, middle-ear balance problems such as dizziness, acute vertigo, and tinnitus are all seen in APS.

A study of endothelial assessment in patients with APS suggests that preclinical atherosclerosis may be an important feature of APS.

Specifically, the results of high-resolution ultrasound studies showed significantly reduced endothelium-dependent, flow-mediated dilatation and increased carotid intima media thickness among 90 APS patients, compared with 90 age-, sex-, and cardiovascular risk factor–matched aPL-negative controls (Arthritis Rheum. 2006;54:557-8).

“We still don't know the exact mechanism for thrombosis. Studies have shown that aPL antibodies alter platelets, clotting proteins, and the blood vessel lining, but how each of these mechanisms contributes to APS is uncertain,” he said. “We also don't understand why patients get better when we anticoagulate.”

Disclosures: Dr. Hughes had no financial disclosures relative to his presentation.

DESTIN, FLA. — The clinical spectrum of antiphospholipid syndrome has broadened substantially to include a range of clinical manifestations, including heart valve disease, livedo reticularis, thrombocytopenia, stroke, migraine, seizures, and cognitive dysfunction.

The potential cardiovascular and neurologic complications of antiphospholipid syndrome are especially concerning, said Dr. Graham Hughes, who along with his colleagues first described the prothrombotic condition in 1983.

“The clinical map is still being charted, and a number of recent clinical observations have added to our understanding of it,” said Dr. Hughes, professor of medicine at St. Thomas Hospital in London and director of the London Lupus Centre at London Bridge Hospital.

Antiphospholipid syndrome (APS), also called Hughes syndrome, is generally recognized as an autoimmune condition characterized by arterial and venous vascular thromboses and pregnancy complications in the presence of antiphospholipid (aPL) antibodies.

Dr. Hughes discussed several recent studies that argue for greater awareness, screening, and diagnosis of APS in primary care.

“We are seeing that APS is a major cause of heart attack, stroke, miscarriage, cognitive dysfunction, movement disorders, epilepsy and other conditions. There is a huge cost to delaying the diagnosis.”

If the syndrome is suspected, he said, “it's easy to diagnose and highly treatable,” primarily with aspirin, heparin, or warfarin.

A study of 344 acute coronary syndrome patients showed that 40% of the patients were aPL positive in one or more tests (Am. J. Clin. Pathol. 2009;132:613-20).

Additionally, in a report published in Lancet Neurology, the study population of women younger than 50 years of age who had experienced myocardial infarction or stroke had five times the risk of MI and a 40-fold increase in the risk of stroke, compared with matched healthy controls (Lancet Neurol. 2009;8:998-1005).

“The brain appears to be particularly susceptible to the syndrome,” Dr. Hughes stated, noting that, in addition to stroke—which is now “internationally recognized as an important manifestation” of APS—other neurologic presentations include migraine, memory loss, movement disorders, atypical multiple sclerosis, and epilepsy.

Importantly, new data suggest that a clinical link between migraine, stroke, and APS “is a very real possibility,” Dr. Hughes reported.

“Migraine and stroke are both prominent features of APS, and our own clinical experience with lupus patients at St. Thomas Hospital has been that many younger individuals with APS who have had a stroke—especially females—report a history of migraines prior to the stroke and a dramatic improvement in migraine during anticoagulation treatment,” he said. “Although the evidence is largely anecdotal, it may be that APS is a significant missing link between migraine and stroke.” In fact, he added, “it is now our practice in patients with [APS] and severe migraine attacks to use low-molecular-weight heparin to treat migraine and, potentially, avert stroke.”

In addition to rheumatologists, cardiologists, and neurologists, “more and more specialists are seeing patients with APS, including orthopedists and ear, nose, and throat surgeons,” Dr. Hughes noted, explaining that prospective cohort and case studies have demonstrated an association between aPL antibodies and avascular necrosis, often presenting as nontraumatic metatarsal, rib, and other fractures in individuals with no history of osteoporosis; in addition, middle-ear balance problems such as dizziness, acute vertigo, and tinnitus are all seen in APS.

A study of endothelial assessment in patients with APS suggests that preclinical atherosclerosis may be an important feature of APS.

Specifically, the results of high-resolution ultrasound studies showed significantly reduced endothelium-dependent, flow-mediated dilatation and increased carotid intima media thickness among 90 APS patients, compared with 90 age-, sex-, and cardiovascular risk factor–matched aPL-negative controls (Arthritis Rheum. 2006;54:557-8).

“We still don't know the exact mechanism for thrombosis. Studies have shown that aPL antibodies alter platelets, clotting proteins, and the blood vessel lining, but how each of these mechanisms contributes to APS is uncertain,” he said. “We also don't understand why patients get better when we anticoagulate.”

Disclosures: Dr. Hughes had no financial disclosures relative to his presentation.