Outcome, & prognosis in 273 patients with PSC: A single center study.

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Am J Gastroenterol. 2007 Jan;102(1):107-14.

Characterization, outcome, and

prognosis in 273 patients with primary sclerosing cholangitis: A single center study.

Tischendorf JJ, Hecker H, Kruger M, Manns MP, Meier PN.

Department of Gastroenterology, Hepatology, &

Endocrinology, Medical School of Hannover, Hannover, Germany.

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. PMID: 17037993 [PubMed

- indexed for MEDLINE]

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