CNS Drugs

, Volume 25, Issue 9, pp 721–736

Neuropsychiatric Manifestations in Systemic Lupus Erythematosus

Epidemiology, Pathophysiology and Management
  • Mariana Postal
  • Lilian T. L. Costallat
  • Simone Appenzeller
Therapy In Practice

DOI: 10.2165/11591670-000000000-00000

Cite this article as:
Postal, M., Costallat, L.T.L. & Appenzeller, S. CNS Drugs (2011) 25: 721. doi:10.2165/11591670-000000000-00000

Abstract

Systemic lupus erythematosus (SLE) is a relapsing-remitting autoimmune disease with CNS involvement occurring in up to 75% of patients. However, the frequency of neuropsychiatric manifestations in SLE studies varies widely, depending on the type of manifestations included and the method used for evaluation. CNS involvement may be considered primary if directly related to SLE activity in the CNS or secondary when related to treatment, infections, metabolic abnormalities or other systemic manifestations such as uraemia and hypertension. The pathogenesis of neuropsychiatric SLE is as yet unknown, though numerous autoantibodies and cytokines have been suggested as possible mediators. However, independent of the aetiology of the insult, the final common pathway in neuropsychiatric SLE is the involvement of the cerebral microvasculature.

The diagnosis of primary CNS involvement by SLE is often difficult, as both focal and diffuse manifestations may occur and there is no gold standard for diagnosis. A high index of clinical suspicion, in addition to laboratory and neuroimaging findings may support the diagnosis. Treatment is mostly empirical, although one randomized controlled trial has shown that cyclophosphamide in addition to methylprednisolone is superior to methylprednisolone alone in severe neuropsychiatric SLE.

Copyright information

© Adis Data Information BV 2011

Authors and Affiliations

  • Mariana Postal
    • 1
  • Lilian T. L. Costallat
    • 1
  • Simone Appenzeller
    • 1
  1. 1.Department of Medicine, Rheumatology Unit, Faculty of Medical ScienceState University of CampinasCampinasBrazil

Personalised recommendations