Gastroenterology 1991 Jun;100(6):1710-7

Natural history and prognostic variables in primary sclerosing cholangitis

Farrant JM, Hayllar KM, Wilkinson ML, Karani J, Portmann BC, Westaby D, Williams
R
Department of Radiology, King's College Hospital, Denmark Hill, London, England

Abstract
The clinical features at the time of presentation and the outcome in 126
patients with primary sclerosing cholangitis were studied to clarify the natural
history and prognosis in symptomatic and asymptomatic individuals. The median
age of the patients at the time of presentation was 36 years, 62% were male, and
16% were asymptomatic. The median follow-up from time of presentation was 5.8
years. There were more patients who had liver transplants (21%) than patients
who died of liver-related disease (16%); the estimated median survival to these
end points was 12 years. Cholangiocarcinoma was found in 8 patients and in 23%
of those undergoing liver transplantation. Asymptomatic patients had milder
disease than symptomatic patients, but in a univariate analysis the presence of
symptoms was not prognostically significant. On multivariate analysis, the
following independent prognostic factors were found: hepatomegaly, splenomegaly,
serum alkaline phosphatase, histological stage, and age. These features were
combined to produce a prognostic model that should be valuable in the
stratification of patients in clinical trials and in the timing of liver
transplantation, particularly in those patients seen soon after presentation