Hepatology 1989 Oct;10(4):430-6

Primary sclerosing cholangitis: natural history, prognostic factors and survival
analysis

Wiesner RH, Grambsch PM, Dickson ER, Ludwig J, MacCarty RL, Hunter EB, Fleming
TR, Fisher LD, Beaver SJ, LaRusso NF
Department of Diagnostic Radiology, Mayo Clinic, Rochester, Minnesota 55905

Abstract
The natural history of primary sclerosing cholangitis was assessed in 174
patients; 37 were asymptomatic and 137 had symptoms related to underlying liver
disease. At the time of diagnosis, the mean age was 39.9 years, 66% of the
primary sclerosing cholangitis patients were male and 71% had associated
inflammatory bowel disease, most commonly chronic ulcerative colitis. Long-term
follow-up (mean: 6.0 years; range: 2.7 to 15.5 years) was available in all
patients. During follow-up, 59 (34%) of the patients died: 55 in the symptomatic
group and four in the asymptomatic group. Median survival from the time of
diagnosis of primary sclerosing cholangitis at the Mayo Clinic was 11.9 years.
Survival in the asymptomatic group was significantly decreased compared with
that in a control population matched for age, race and sex. Multivariate
analysis (Cox proportional hazards regression modeling) revealed that age, serum
bilirubin concentration, blood hemoglobin concentration, presence or absence of
inflammatory bowel disease and histologic stage on liver biopsy were independent
predictors of high risk of dying. The development of a multivariate statistical
survival model is a major step in identifying individual primary sclerosing
cholangitis patients at low, moderate and high risk of dying. Such models will
be useful for stratifying patients in therapeutic trials, in patient counseling
and in patient selection and timing of liver transplantation