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

Arthritis Rheumatol. 2019 Jan;71(1):33-42. doi: 10.1002/art.40591. Epub 2018 Nov 24.


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.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Autoantibodies / immunology
  • Blood-Brain Barrier / metabolism
  • Brain / diagnostic imaging
  • Brain / immunology
  • Brain / physiopathology*
  • Brain Ischemia / physiopathology
  • Cognitive Dysfunction / immunology
  • Cognitive Dysfunction / physiopathology*
  • Cognitive Dysfunction / therapy
  • Cytokines / immunology
  • Humans
  • Inflammation / immunology
  • Lupus Vasculitis, Central Nervous System / diagnostic imaging
  • Lupus Vasculitis, Central Nervous System / immunology
  • Lupus Vasculitis, Central Nervous System / physiopathology*
  • Lupus Vasculitis, Central Nervous System / therapy
  • Magnetic Resonance Imaging
  • Positron-Emission Tomography


  • Autoantibodies
  • Cytokines