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Abstract
Neuropsychiatric lupus affects above 50% of patients with systemic lupus erythematosus and may span from mild symptoms to acute devastating life-threatening ones. Owing to the clinical variability, most pharmacological data rely on small, uncontrolled trials and case reports. The mainstay of therapy relies on immune-suppression by glucocorticoids, in adjunction with cyclophosphamide or anti-B-cell therapy, in moderate to severe cases. In selected scenarios (e.g., chorea) intravenous immunoglobulin or plasmapheresis may be effective. Anticoagulation is warranted if anti-phospholipid antibodies are present. In parallel there may be a need for symptomatic treatment such as anti-epileptic or anti-depressive treatments, etc. In the future, more studies addressed to assess pathogenesis and preferred treatments of specific manifestations are needed in order to personalize treatments.
1. First exclude non-systemic lupus erythematosus intercurrent infectious illness, medication side effects (especially steroids), and psychosocial- or functional-related conditions.
2. In neuropsychiatric systemic lupus erythematosus (NPSLE), glucocorticoids are the main treatment, sparing agents are often used (e.g. azathioprine).
3. In the moderate to severe cases, cyclophosphamide or B-cell depletion is needed in adjunct to glucocorticoids.
4. In selected cases adding anticoagulants (when anti-phospholipid antibodies are present), plasmapheresis, or intravenous immunoglobulin is effective.
5. There may be a need for symptomatic treatments in parallel to immunosuppressive ones (anti-depressive, anti-anxiolytic, ant-epileptic, etc.)
6. Current treatments are based on sparse evidence; further randomized controlled trials must be done in order to gain a better understanding of available treatment options.
7. The mechanisms behind the 19 different manifestations of NPSLE must be better understood in order to have more focused treatment.
Financial & competing interests’ disclosure
The author has no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.