Characterization, Outcome, & Prognosis in 273 Patients with PSC: A Single Center Study

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Characterization,

Outcome, and Prognosis in 273 Patients with Primary Sclerosing Cholangitis: A Single Center Study

Authors:

Tischendorf, Jens J.

W.; Hecker, Hartmut; Krüger, ; Manns,

P.; Meier, N

Source:

The American Journal

of Gastroenterology, January 2007  Publisher: Blackwell

Publishing

Abstract:

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 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.  (Am J Gastroenterol 2007;102:107-114)

Document Type: Research article   DOI: 10.1111/j.1572-0241.2006.00872.x

Affiliations: 1: Institute of Biometry, Medical School

of Hannover, Hannover,

Germany 2: Gastroenterology,

Hepatology, and Endocrinology

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