title: Immune-mediated diseases in primary sclerosing cholangitis.
abstract: BACKGROUND
  Primary sclerosing cholangitis is a chronic cholestatic liver disease. An immune aetiology is suggested by associations between PSC and inflammatory bowel disease. Data on concomitant prevalence of other immune-mediated diseases is limited.
  
  
  OBJECTIVE
  To assess the prevalence of concomitant immune-mediated diseases and the impact on disease outcome in PSC.
  
  
  METHODS
  We included 241 patients and retrospectively reviewed medical charts.
  
  
  RESULTS
  Altogether 172 (71.4%) patients had concomitant immune-mediated disease, including IBD (149, 61.8%), autoimmune hepatitis (15, 6.2%) and other immune-mediated diseases (47, 19.5%). Thirty nine patients (22.7%) had more than one immune-mediated disease other than PSC. Most frequent extrahepatic non-IBD immune-mediated diseases were sarcoidosis, thyroid disease, and type I diabetes mellitus. Age at PSC diagnosis was lower in patients with IBD. In patients with other immune-mediated diseases than autoimmune hepatitis or IBD, age at PSC diagnosis was higher. Younger age at diagnosis and concomitant IBD related to longer survival till death or liver transplantation.
  
  
  CONCLUSIONS
  In a large PSC population, a high prevalence of concomitant immune-mediated diseases was found. IBD occurred more often in early-acquired PSC, and the other immune-mediated diseases more often in later-acquired PSC. No effect on outcome was found for non-IBD immune mediated disease.
authors.affiliation: Department of Gastroenterology and Hepatology, University Medical Center Groningen, University of Groningen, PO Box 30001, 9700 RB Groningen, The Netherlands.
authors.forename: Laetitia E
authors.initials: LE
authors.lastname: Lamberts
authors.forename: Marcel
authors.initials: M
authors.lastname: Janse
authors.forename: Elizabeth B
authors.initials: EB
authors.lastname: Haagsma
authors.forename: Arie P
authors.initials: AP
authors.lastname: van den Berg
authors.forename: Rinse K
authors.initials: RK
authors.lastname: Weersma
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000368:Aged; D001650:Bile Duct Neoplasms / Q000175:diagnosis; D001653:Bile Ducts, Intrahepatic; D002648:Child; D002675:Child, Preschool; D018281:Cholangiocarcinoma / Q000175:diagnosis; D015209:Cholangitis, Sclerosing / Q000150:complications* / Q000175:diagnosis / Q000276:immunology*; D003922:Diabetes Mellitus, Type 1 / Q000150:complications* / Q000175:diagnosis / Q000276:immunology; D005260:Female; D019693:Hepatitis, Autoimmune / Q000150:complications*; D006801:Humans; D015212:Inflammatory Bowel Diseases / Q000150:complications* / Q000175:diagnosis / Q000276:immunology; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D012189:Retrospective Studies; D012507:Sarcoidosis / Q000150:complications* / Q000175:diagnosis / Q000276:immunology; D013959:Thyroid Diseases / Q000150:complications* / Q000175:diagnosis / Q000276:immunology; D055815:Young Adult