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Rev Med Interne. 2012 Sep;33(9):491-502. doi: 10.1016/j.revmed.2012.03.356. Epub 2012 May 11.

[Neuropsychiatric systemic lupus erythematosus (1st part). Cases definitions and diagnosis and treatment of central nervous system and psychiatric manifestations of systemic lupus erythematosus].

[Article in French]

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  • 1Service de médecine interne, université de Lille Nord-de-France, centre de référence maladies auto-immunes rares (sclérodermie), hôpital Claude-Huriez, CHRU de Lille, 1, rue Michel-Polonovski, 59037 Lille, France; EA2686, Institut d'immunologie, université Lille Nord-de-France, faculté de médecine H.-Warembourg, 59037 Lille, France.


Systemic lupus erythematosus (SLE) is an autoimmune disease, which primarily affects skin and joints. Peripheral neurologic syndrome and central nervous system (CNS) manifestations are common in lupus patients but are not always attributable to lupus itself. A classification, published in 1999 by the American College of Rheumatology (ACR) research committee, described 12 CNS syndromes and seven peripheral neurologic syndromes compatible with "neuropsychiatric systemic lupus erythematosus" (NPSLE). Despite this consensus, studies which have been published since 1999 have reported a prevalence of NPSLE varying from 20 to 97 %, which shows the diagnosis difficulty and the heterogeneity of neuropsychiatric manifestations in SLE. In order to understand the limits of this classification, we propose in this first part an exhaustive review of publications describing neuropsychiatric manifestations according to the ACR 1999 classification. We also detail case definitions, prevalence and risk factors, clinical characteristics and diagnosis of each lupus-related psychiatric and CNS manifestation.

Copyright © 2012 Société nationale française de médecine interne (SNFMI). Published by Elsevier SAS. All rights reserved.

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