title: Characterization, outcome, and prognosis in 273 patients with primary sclerosing cholangitis: A single center study.
abstract: OBJECTIVE
  Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease with varying severity and progression. This study describes the natural history of PSC patients and evaluates the prognostic significance of clinical, biochemical, and cholangiographic findings constructing a novel prognostic model.
  
  
  METHODS
  A population of 273 German PSC patients was studied with a median follow-up time of 76 months (range 1-280 months). Survival curves were analyzed by the Kaplan-Meier method, and prognostic significance of clinical, biochemical, and cholangiographic features recorded at the time of diagnosis was evaluated by multivariate analysis using Cox proportional-hazards regression models.
  
  
  RESULTS
  The estimated median survival from the time of diagnosis to death or time of liver transplantation was 9.6 yr. One hundred eight (39.6%) patients underwent liver transplantation. Hepatobiliary malignancies were found in 39 (14.3%) patients of the entire PSC population. Age, low albumin, persistent bilirubin elevation longer than 3 months, hepatomegaly, splenomegaly, dominant bile duct stenosis, and intra- and extrahepatic ductal changes at the time of diagnosis were found to be independent risk factors correlating with poor prognosis and were used to construct a new prognostic model.
  
  
  CONCLUSIONS
  A persistent bilirubin elevation for longer than 3 months from the time of diagnosis could be identified as a novel marker correlating with a poor outcome. A new prognostic model was developed to predict progression of PSC, which may be useful in timing of liver transplantation.
authors.affiliation: Department of Gastroenterology, Hepatology, and Endocrinology, Medical School of Hannover, Hannover, Germany.
authors.forename: Jens J W
authors.initials: JJ
authors.lastname: Tischendorf
authors.forename: Hartmut
authors.initials: H
authors.lastname: Hecker
authors.forename: Martin
authors.initials: M
authors.lastname: Krüger
authors.forename: Michael P
authors.initials: MP
authors.lastname: Manns
authors.forename: Peter N
authors.initials: PN
authors.lastname: Meier
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D015209:Cholangitis, Sclerosing / Q000175:diagnosis / Q000401:mortality / Q000628:therapy*; D005260:Female; D005858:Germany / Q000453:epidemiology; D006801:Humans; D016031:Liver Transplantation; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D016016:Proportional Hazards Models; D015996:Survival Rate; D016896:Treatment Outcome