EULAR 2009: New EULAR Recommendations for Neuropsychiatric Manifestations in SLE

Alice Goodman

June 19, 2009

June 19, 2009 (Copenhagen, Denmark) — New recommendations for the diagnosis and management of systemic lupus erythematosus (SLE) with neuropsychiatric manifestations were presented here at EULAR 2009: The Annual European Congress of Rheumatology. The recommendations are based on a systematic review and expert opinion and include diagnostic and therapeutic algorithms for the more common problematic neuropsychiatric manifestations of SLE. An article presenting the new recommendations will be submitted to the Annals of Rheumatic Diseases next month.

"Neuropsychiatric manifestations of SLE are frightening for physicians and patients and can be complex," said George Bertsias, MD, from the University of Crete in Greece. Dr. Bertsias presented the new guidelines on behalf of the EULAR Standing Committee for Clinical Affairs Task Force on SLE.

Neuropsychiatric manifestations of SLE "usually occur in relatively young women of productive ages. There is heterogeneity in the way physicians approach diagnosis, and sometimes they are not clear about which tests to use. These new recommendations will hopefully facilitate medical care in SLE patients without restricting physicians' autonomy," Dr. Bertsias said.

The task force includes 24 experts representing most countries in Europe. Lupus experts, 2 neurologists, and a clinical epidemiologist worked together to develop 14 evidence-based recommendations (also called general statements).

About 10% to 15% of SLE patients have a lifetime risk for neuropsychiatric manifestations, and severe manifestations (but not mild ones) correlate with the severity of lupus, Dr. Bertsias explained.

"The very important severe manifestations occur in about 10% [of patients] and include stroke, seizures, acute confusion, and cognitive impairment. Milder manifestations include headache and anxiety," he said.

About 50% of SLE patients who develop neuropsychiatric manifestations do so within a year of being diagnosed with lupus. At the time these manifestations emerge, patients have active lupus. Risk factors identified in the recommendations include uncontrolled SLE, a history of neuropsychiatric manifestations associated with SLE, and the presence of antiphospholipid antibodies (APS).

Exclusion of Other Causes Needed

To make the diagnosis of neuropsychiatric manifestations of SLE, other causes must be excluded, such as central nervous system infection. Diagnostic tests can include lumbar puncture, cerebrospinal fluid (CSF) analysis, neuropsychological cognitive function tests, nerve conduction studies, and neuroimaging with magnetic resonance imaging (MRI). Structural MRI and functional neuroimaging should be coupled.

"The specific battery of tests depends on the manifestation," Dr. Bertsias said.

Corticosteroids and immunosuppressants are recommended as treatment for neuropsychiatric manifestations that reflect an immune/inflammatory process, such as acute confusional state, aseptic meningitis, myelitis, cranial and peripheral neuropathies, and psychosis. Antiplatelet/anticoagulation therapy is indicated for manifestations related to APS, particularly thrombotic cerebrovascular disease. Symptomatic therapies should also be considered.

Eight of the recommendations/statements focus on common specific severe neuropsychiatric manifestations.

At EULAR, Dr. Bertsias showed attendees an algorithm for managing suspected cognitive dysfunction as an example of the types of algorithms that will be part of the published article; it included assessment of risk factors, screening, confirmation of the diagnosis, necessary tests, and suggested treatment.

These recommendations will be important for rheumatologists and neurologists, who are often called upon for consultation of patients with neuropsychiatric manifestations, Dr. Bertsias said.

"It is difficult to [distinguish] psychiatric conditions that occur in the general population from those that are related to SLE," Arthur Kavanaugh, MD, from the University of California at San Diego, pointed out. "Even with neuroimaging and CSF testing, it is still difficult to differentiate infection, lupus-related neuropsychiatric manifestations, and some psychiatric manifestations.

"These recommendations will be a welcome addition for rheumatologists," he said.

Dr. Bertsias has disclosed no relevant financial relationships. Dr. Kavanaugh has received research support from Centocor.

EULAR 2009: The Annual European Congress of Rheumatology: Abstract OP-0276. Presented June 11, 2009.

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