title: Survival and risk of cholangiocarcinoma in patients with primary sclerosing cholangitis. A population-based study.
abstract: BACKGROUND
  Endoscopic retrograde cholangiopancreatography was introduced in the early 1970s, making a more reliable diagnosis of primary sclerosing cholangitis (PSC) possible. Since then decreased survival and increased risk of cholangiocarcinoma have been reported. However, no population-based studies have quantified these outcomes.
  
  
  METHODS
  A population-based cohort of 125 patients with a verified PSC diagnosis was followed up through linkage to the Swedish Death Registry and the Swedish Cancer Registry for occurrence of death and cholangiocarcinoma.
  
  
  RESULTS
  The diagnosis of PSC was associated with a substantially decreased survival, with an overall 10-year survival of 68.8%. Patients with a diagnosis of inflammatory bowel disease (IBD) had a somewhat better prognosis, 71.8%, compared with 60% for patients without. Fourteen subsequent cholangiocarcinomas yielded a cumulative risk of 11.2% 10 years after diagnosis. Sex, duration of IBD, and colectomy influenced neither the survival nor the cholangiocarcinoma risk.
  
  
  CONCLUSIONS
  Patients with PSC have a substantially decreased survival, which is most pronounced among patients without IBD.
authors.affiliation: Dept. of Cancer Epidemiology, University Hospital, Uppsala, Sweden.
authors.forename: D
authors.initials: D
authors.lastname: Kornfeld
authors.forename: A
authors.initials: A
authors.lastname: Ekbom
authors.forename: T
authors.initials: T
authors.lastname: Ihre
mesh_terms: D000206:Actuarial Analysis; D000328:Adult; D001650:Bile Duct Neoplasms / Q000453:epidemiology*; D001653:Bile Ducts, Intrahepatic; D002423:Cause of Death; D018281:Cholangiocarcinoma / Q000453:epidemiology*; D002760:Cholangiopancreatography, Endoscopic Retrograde; D015209:Cholangitis, Sclerosing / Q000150:complications / Q000000981:diagnostic imaging / Q000401:mortality*; D015331:Cohort Studies; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012042:Registries; D012307:Risk Factors; D013548:Sweden / Q000453:epidemiology