EULAR Issues Recommendations for Managing SLE's Neuropsychiatric Effects

Neuropsychiatric manifestations in patients with systemic lupus erythematosus should be evaluated and treated initially as they would in patients without the autoimmune disease before being attributed to the condition, according to new recommendations put forth by a European League Against Rheumatism task force.

Using an evidence-based approach followed by expert consensus, the task force gathered relevant information on neuropsychiatric systemic lupus erythematosus (NPSLE), including prevalence, risk factors, diagnosis, monitoring, therapy, and prognosis and developed management recommendations in an effort to alleviate the persistent diagnostic and therapeutic challenges associated with the condition.

[Check out our coverage from the American College of Rheumatology's annual meeting.]

Based on a systematic literature and subsequent categorization of the quality of the evidence, lead author Dr. George K. Bertsias of the University of Crete in Heraklion and his colleagues on the task force of the EULAR standing committee for clinical affairs, determined that most NPSLE events occur at disease onset or within the first year of onset, and that while manifestations such as headache, mood disorders, anxiety, and mild cognitive dysfunction are common, they "do not usually reflect overt central nervous system lupus activity."

After excluding the mild manifestations noted above, cerebrovascular disease and seizures are the most common neuropsychiatric complications of SLE, with a cumulative incidence of 5%-15% and severe cognitive dysfunction, major depression, acute confusional state, peripheral nervous system disorders, and psychosis are all relatively uncommon, occurring in approximately 1%-5% of all SLE patients, the authors wrote (Ann. Rheum. Dis. 2010.;69:2074-82 [doi:10.1136/ard.2010.130476]).

In all SLE patients with new or unexplained symptoms or signs suggestive of neuropsychiatric disease, "the initial diagnostic work-up should be similar to that in non-SLE patients presenting with the same manifestations," the guidelines state. This is because the complications are generally not distinctive and could occur in the absence of SLE, according to the authors. Depending on the manifestation, the work-up may include lumbar puncture and cerebrospinal fluid analysis, EEG, neuropsychological assessment of cognitive function, nerve conduction studies, and neuroimaging, they wrote.

Possible causative factors for NPSLE include vascular injury of intracranial vessels, antibodies to neuronal antigens, ribosomes, and phospholipids-associated proteins, and inflammatory mediators, and risk factors include general SLE activity or damage, past or concurrent NPSLE, and moderate to high titers of persistently positive antiphospholipid antibodies, the authors wrote, noting that "these observations provide the rationale for primary and secondary prevention strategies."

Such strategies include glucocorticoids and immunosuppressant therapy "when the underlying pathogenesis is considered primarily inflammatory (such as in optic neuritis, transverse myelitis, or psychosis) or there is evidence of generalized SLE activity;" antiplatelet/antithrombotic therapy for moderate to high antiphospholipid antibodies or other features of antiphospholipid antibody syndrome, particularly thrombotic cerebrovascular disease; and symptomatic interventions and treatment of non-SLE factors as indicated, according to the guidelines. The efficacy of all of the interventions should be further defined in randomized controlled trial, the authors stressed.

The EULAR recommendations on NPSLE will be updated every 3 years, according to the authors, "with the inclusion of patients and individuals from other relevant professions, and the development of tools that will facilitate the dissemination and implementation of the recommendations."

This study was funded by the European League Against Rheumatism. The authors did not provide financial disclosures.

Neuropsychiatric manifestations in patients with systemic lupus erythematosus should be evaluated and treated initially as they would in patients without the autoimmune disease before being attributed to the condition, according to new recommendations put forth by a European League Against Rheumatism task force.

Using an evidence-based approach followed by expert consensus, the task force gathered relevant information on neuropsychiatric systemic lupus erythematosus (NPSLE), including prevalence, risk factors, diagnosis, monitoring, therapy, and prognosis and developed management recommendations in an effort to alleviate the persistent diagnostic and therapeutic challenges associated with the condition.

[Check out our coverage from the American College of Rheumatology's annual meeting.]

Based on a systematic literature and subsequent categorization of the quality of the evidence, lead author Dr. George K. Bertsias of the University of Crete in Heraklion and his colleagues on the task force of the EULAR standing committee for clinical affairs, determined that most NPSLE events occur at disease onset or within the first year of onset, and that while manifestations such as headache, mood disorders, anxiety, and mild cognitive dysfunction are common, they "do not usually reflect overt central nervous system lupus activity."

After excluding the mild manifestations noted above, cerebrovascular disease and seizures are the most common neuropsychiatric complications of SLE, with a cumulative incidence of 5%-15% and severe cognitive dysfunction, major depression, acute confusional state, peripheral nervous system disorders, and psychosis are all relatively uncommon, occurring in approximately 1%-5% of all SLE patients, the authors wrote (Ann. Rheum. Dis. 2010.;69:2074-82 [doi:10.1136/ard.2010.130476]).

In all SLE patients with new or unexplained symptoms or signs suggestive of neuropsychiatric disease, "the initial diagnostic work-up should be similar to that in non-SLE patients presenting with the same manifestations," the guidelines state. This is because the complications are generally not distinctive and could occur in the absence of SLE, according to the authors. Depending on the manifestation, the work-up may include lumbar puncture and cerebrospinal fluid analysis, EEG, neuropsychological assessment of cognitive function, nerve conduction studies, and neuroimaging, they wrote.

Possible causative factors for NPSLE include vascular injury of intracranial vessels, antibodies to neuronal antigens, ribosomes, and phospholipids-associated proteins, and inflammatory mediators, and risk factors include general SLE activity or damage, past or concurrent NPSLE, and moderate to high titers of persistently positive antiphospholipid antibodies, the authors wrote, noting that "these observations provide the rationale for primary and secondary prevention strategies."

Such strategies include glucocorticoids and immunosuppressant therapy "when the underlying pathogenesis is considered primarily inflammatory (such as in optic neuritis, transverse myelitis, or psychosis) or there is evidence of generalized SLE activity;" antiplatelet/antithrombotic therapy for moderate to high antiphospholipid antibodies or other features of antiphospholipid antibody syndrome, particularly thrombotic cerebrovascular disease; and symptomatic interventions and treatment of non-SLE factors as indicated, according to the guidelines. The efficacy of all of the interventions should be further defined in randomized controlled trial, the authors stressed.

The EULAR recommendations on NPSLE will be updated every 3 years, according to the authors, "with the inclusion of patients and individuals from other relevant professions, and the development of tools that will facilitate the dissemination and implementation of the recommendations."

This study was funded by the European League Against Rheumatism. The authors did not provide financial disclosures.

Neuropsychiatric manifestations in patients with systemic lupus erythematosus should be evaluated and treated initially as they would in patients without the autoimmune disease before being attributed to the condition, according to new recommendations put forth by a European League Against Rheumatism task force.

Using an evidence-based approach followed by expert consensus, the task force gathered relevant information on neuropsychiatric systemic lupus erythematosus (NPSLE), including prevalence, risk factors, diagnosis, monitoring, therapy, and prognosis and developed management recommendations in an effort to alleviate the persistent diagnostic and therapeutic challenges associated with the condition.

[Check out our coverage from the American College of Rheumatology's annual meeting.]

Based on a systematic literature and subsequent categorization of the quality of the evidence, lead author Dr. George K. Bertsias of the University of Crete in Heraklion and his colleagues on the task force of the EULAR standing committee for clinical affairs, determined that most NPSLE events occur at disease onset or within the first year of onset, and that while manifestations such as headache, mood disorders, anxiety, and mild cognitive dysfunction are common, they "do not usually reflect overt central nervous system lupus activity."

After excluding the mild manifestations noted above, cerebrovascular disease and seizures are the most common neuropsychiatric complications of SLE, with a cumulative incidence of 5%-15% and severe cognitive dysfunction, major depression, acute confusional state, peripheral nervous system disorders, and psychosis are all relatively uncommon, occurring in approximately 1%-5% of all SLE patients, the authors wrote (Ann. Rheum. Dis. 2010.;69:2074-82 [doi:10.1136/ard.2010.130476]).

In all SLE patients with new or unexplained symptoms or signs suggestive of neuropsychiatric disease, "the initial diagnostic work-up should be similar to that in non-SLE patients presenting with the same manifestations," the guidelines state. This is because the complications are generally not distinctive and could occur in the absence of SLE, according to the authors. Depending on the manifestation, the work-up may include lumbar puncture and cerebrospinal fluid analysis, EEG, neuropsychological assessment of cognitive function, nerve conduction studies, and neuroimaging, they wrote.

Possible causative factors for NPSLE include vascular injury of intracranial vessels, antibodies to neuronal antigens, ribosomes, and phospholipids-associated proteins, and inflammatory mediators, and risk factors include general SLE activity or damage, past or concurrent NPSLE, and moderate to high titers of persistently positive antiphospholipid antibodies, the authors wrote, noting that "these observations provide the rationale for primary and secondary prevention strategies."

Such strategies include glucocorticoids and immunosuppressant therapy "when the underlying pathogenesis is considered primarily inflammatory (such as in optic neuritis, transverse myelitis, or psychosis) or there is evidence of generalized SLE activity;" antiplatelet/antithrombotic therapy for moderate to high antiphospholipid antibodies or other features of antiphospholipid antibody syndrome, particularly thrombotic cerebrovascular disease; and symptomatic interventions and treatment of non-SLE factors as indicated, according to the guidelines. The efficacy of all of the interventions should be further defined in randomized controlled trial, the authors stressed.

The EULAR recommendations on NPSLE will be updated every 3 years, according to the authors, "with the inclusion of patients and individuals from other relevant professions, and the development of tools that will facilitate the dissemination and implementation of the recommendations."

This study was funded by the European League Against Rheumatism. The authors did not provide financial disclosures.