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Pathogenesis, diagnosis and management of neuropsychiatric SLE manifestations

Nature Reviews Rheumatology volume 6, pages 358–367 (2010)Cite this article

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Abstract

Neuropsychiatric events are common in patients with systemic lupus erythematosus (SLE), but less than one-third of these events can be directly attributed to SLE. Increased generalized SLE disease activity or damage, previous or concurrent major neuropsychiatric SLE (NPSLE) events, and persistently positive moderate-to-high antiphospholipid antibody titers are established risk factors, and their presence could facilitate proper attribution to the disease itself. Diagnostic evaluation is guided by the presenting manifestation; MRI is used to visualize brain or spinal pathologies. For neuropsychiatric events believed to reflect an immune or inflammatory process, or when these events occur in the context of active generalized disease, evidence (primarily from uncontrolled studies) supports the use of glucocorticoids alone or in combination with immunosuppressive therapy. Antiplatelet and/or anticoagulation therapy is recommended for NPSLE manifestations related to antiphospholipid antibodies, especially for thrombotic cerebrovascular disease. For the future, we anticipate that novel biomarkers and advanced neuroimaging tests will better define the underlying pathologic mechanisms of SLE-related neuropsychiatric disease, and help guide therapeutic decisions.

Key Points

  • Systemic lupus erythematosus (SLE)-associated neuropsychiatric events occur in 30–40% of patients with this disease, often at disease onset or within the first 2 years after diagnosis

  • Mild neuropsychiatric syndromes, such as headache, anxiety, mild depression, mild cognitive dysfunction, and polyneuropathy without electrophysiologic confirmation, are common, but are not usually related to SLE

  • Risk factors for neuropsychiatric SLE (NPSLE) include persistently positive, moderate-to-high titers of antiphospholipid antibodies (aPL), previous or concurrent major NPSLE events, and increased SLE activity or damage

  • Initial diagnostic evaluation should be similar to that in non-SLE patients, aiming to exclude secondary causes of neuropsychiatric events; MRI is the preferred imaging test for visualizing brain and spinal pathologies

  • Management involves symptomatic therapies and treatment of aggravating factors where applicable: immunosuppressive therapy is indicated for inflammatory NPSLE or when generalized SLE activity is present; anticoagulation/antiplatelet therapy is recommended for aPL-associated manifestations

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Figure 1: Algorithms for the diagnosis and management of patients with SLE who present with diffuse neuropsychiatric syndromes.
Figure 2: Algorithms for the diagnosis and management of patients with SLE who present with focal neuropsychiatric syndromes.

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  1. Division of Rheumatology, Department of Internal Medicine, Clinical Immunology, and Allergy, University of Crete School of Medicine, 1 Voutes Street, Heraklion, 71 003, Greece

    George K. Bertsias & Dimitrios T. Boumpas

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Bertsias, G., Boumpas, D. Pathogenesis, diagnosis and management of neuropsychiatric SLE manifestations. Nat Rev Rheumatol 6, 358–367 (2010). https://doi.org/10.1038/nrrheum.2010.62

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