title: Neuropsychiatric manifestations in patients with systemic lupus erythematosus: epidemiology and radiology pointing to an immune-mediated cause.
abstract: BACKGROUND
  Different pathogenetic pathways have been proposed for neuropsychiatric (NP) manifestations in systemic lupus erythematosus (SLE).
  
  
  OBJECTIVE
  To describe the patient characteristics of a large cohort of patients with SLE with NP manifestations (NPSLE) in a single centre and to review whether these and other data are compatible with immune-mediated mechanisms.
  
  
  METHODS
  A total of 212 patients were identified from MRI scans of the brain ordered for suspected NPSLE. Data were collected from the medical records. NP syndromes were classified according to the American College of Rheumatology (ACR) nomenclature and case definitions.
  
  
  RESULTS
  155 patients fulfilled the criteria for SLE. In 102 patients NP manifestations were attributed to SLE itself (primary NPSLE) whereas, in the remaining patients, the NP symptoms were due to other causes. The median age at the time of SLE diagnosis in patients with primary NPSLE was 27.5 years and the median duration prior to NPSLE was 2.8 years. Forty patients (39%) had a NP manifestation in the first year of the disease. Cerebrovascular disease, cognitive dysfunction, seizures and headache were the most prevalent syndromes. In 47% of patients with primary NPSLE the MRI scan of the brain showed no abnormalities.
  
  
  CONCLUSIONS
  Most NP manifestations in SLE occur early in the disease. This finding, as well as data from quantitative imaging studies and recent pathological studies, point to an immune-mediated pathogenesis.
authors.affiliation: Department of Rheumatology, C1-R, Leiden University Medical Center, PO Box 9600, Leiden 2300 RC, The Netherlands. g.m.steup-beekman@lumc.nl
authors.forename: Gerda M
authors.initials: GM
authors.lastname: Steup-Beekman
authors.forename: Elisabeth J M
authors.initials: EJ
authors.lastname: Zirkzee
authors.forename: Danielle
authors.initials: D
authors.lastname: Cohen
authors.forename: Bastiaan M A
authors.initials: BM
authors.lastname: Gahrmann
authors.forename: Bart J
authors.initials: BJ
authors.lastname: Emmer
authors.forename: Stefan C A
authors.initials: SC
authors.lastname: Steens
authors.forename: Eduard L E M
authors.initials: EL
authors.lastname: Bollen
authors.forename: Mark A
authors.initials: MA
authors.lastname: van Buchem
authors.forename: Tom W J
authors.initials: TW
authors.lastname: Huizinga
mesh_terms: D000293:Adolescent; D000328:Adult; D015331:Cohort Studies; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007154:Immune System Diseases / Q000150:complications* / Q000175:diagnosis / Q000453:epidemiology; D020945:Lupus Vasculitis, Central Nervous System / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology*; D008279:Magnetic Resonance Imaging; D008297:Male; D015995:Prevalence; D012189:Retrospective Studies; D055815:Young Adult
chemical_list: D005938:Glucocorticoids