Characterization, outcome, and prognosis in 273 patients with primary sclerosing cholangitis: A single center study

Am J Gastroenterol. 2007 Jan;102(1):107-14. doi: 10.1111/j.1572-0241.2006.00872.x. Epub 2006 Oct 13.


Objectives: 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.

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Child
  • Cholangitis, Sclerosing / diagnosis
  • Cholangitis, Sclerosing / mortality
  • Cholangitis, Sclerosing / therapy*
  • Female
  • Germany / epidemiology
  • Humans
  • Liver Transplantation
  • Male
  • Middle Aged
  • Prognosis
  • Proportional Hazards Models
  • Survival Rate
  • Treatment Outcome