title: Central nervous system involvement in Sjögren's syndrome: unusual, but not unremarkable--clinical, serological characteristics and outcomes in a large cohort of Italian patients.
abstract: OBJECTIVE
  To perform an observational retrospective cross-sectional case-control study to evaluate prevalence, clinical patterns and outcomes of CNS involvement in a large cohort of primary SS (pSS) patients.
  
  
  METHODS
  A total of 424 pSS patients, diagnosed according to the 2002 criteria proposed by the American-European Consensus Group, were checked for CNS involvement after exclusion of secondary causes. Demographic, clinical, seroimmunological data were compared between patients with and without CNS involvement. Neuroimaging data were also analysed.
  
  
  RESULTS
  CNS involvement was detected in 25 (5.8%) patients (24 females and 1 male) both at disease onset (52%) and later (48%) with a mean latency after diagnosis of 7 years. Diffuse (40%), focal/multifocal (36%), multiple sclerosis (MS)-like disease (20%) and isolated optic neuritis (4%) were the most common CNS clinical pictures. Disease duration, lung involvement and decreased C(4) were associated with CNS involvement, while articular manifestations were more frequently observed in patients without neurological complications. Most cases had an acute, often recurrent course with spontaneous remission or only mild neurological impairment.
  
  
  CONCLUSIONS
  CNS involvement represents a rare but not negligible complication of pSS, which may occur with a bimodal temporal pattern, both at onset and later, prompting attention in the differential diagnosis of apparently isolated neurological syndromes. Lung involvement emerged as the strongest risk factor for CNS involvement with a relative risk of 7.9, along with disease duration and decreased C(4).
authors.affiliation: Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Ferrara, Ferrara, Italy.
authors.forename: Alfonso
authors.initials: A
authors.lastname: Massara
authors.forename: Sara
authors.initials: S
authors.lastname: Bonazza
authors.forename: Gabriella
authors.initials: G
authors.lastname: Castellino
authors.forename: Luisa
authors.initials: L
authors.lastname: Caniatti
authors.forename: Francesco
authors.initials: F
authors.lastname: Trotta
authors.forename: Massimo
authors.initials: M
authors.lastname: Borrelli
authors.forename: Luciano
authors.initials: L
authors.lastname: Feggi
authors.forename: Marcello
authors.initials: M
authors.lastname: Govoni
mesh_terms: D000328:Adult; D000368:Aged; D016022:Case-Control Studies; D002493:Central Nervous System Diseases / Q000209:etiology* / Q000473:pathology / Q000503:physiopathology; D015331:Cohort Studies; D018450:Disease Progression; D005260:Female; D006801:Humans; D007558:Italy / Q000453:epidemiology; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D012044:Regression Analysis; D012189:Retrospective Studies; D012859:Sjogren's Syndrome / Q000150:complications* / Q000473:pathology / Q000503:physiopathology; D013997:Time Factors