Systemic lupus erythematosus involving the nervous system: presentation, pathogenesis, and management

Neuropsychiatric syndromes in systemic lupus erythematosus (SLE) have a wide range of severity and phenotype and are fairly common. Presentations are seen involving both central and peripheral nervous systems. Pathogenic mechanisms are thought to include vascular occlusion and hemorrhage, antineuronal antibodies, cytokine effects, choroid plexus dysfunction, neuroendocrine-immune effects, and direct central nervous system (CNS) tissue injury. Work-up includes serum, cerebrospinal fluid (CSF), and imaging studies. Treatment is based on severity and phenotype and includes steroids, anticoagulants, antidepressants, and cytotoxic approaches. Much remains to be done in terms of further elucidation of pathologic processes, correlation of these mechanisms with specific phenotypes, and management of the most severe presentations of neuropsychiatric lupus erythematosus.