Nervous System Disease in Systemic Lupus Erythematosus: Current Status and Future Directions

The American College of Rheumatology's case definitions for 19 neuropsychiatric syndromes in systemic lupus erythematosus ( SLE ) constitute a comprehensive classification of nervous system events in this disease. However, additional strategies are needed to determine whether a neuropsychiatric syndrome is attributable to SLE versus a competing comorbidity. Cognitive function is a clinical surrogate of overall brain health, with applications in both diagnosis and determination of clinical outcomes. Ischemic and inflammatory mechanisms are both key components of the immunopathogenesis of neuropsychiatric SLE ( NPSLE ), including abnormalities of the blood–brain barrier and autoantibody‐mediated production of proinflammatory cytokines. Advances in neuroimaging provide a platform to assess novel disease mechanisms in a noninvasive way. The convergence of more rigorous clinical characterization, validation of biomarkers, and brain neuroimaging provides opportunities to determine the efficacy of novel targeted therapies in the treatment of NPSLE .