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Primary Sclerosing Cholangitis - Prognosis
Last reviewed: 06.07.2025
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In one study, survival time for patients with primary sclerosing cholangitis from diagnosis averaged 11.9 years. In another study, 75% of patients were alive 9 years after diagnosis.
When observing patients with an asymptomatic course of the disease for 6 years, its progression was revealed in 70% of them, with a third developing liver failure.
Although some patients may do well, most develop progressive cholestatic jaundice and liver damage, most notably esophageal variceal bleeding, liver failure, and cholangiocarcinoma.
The prognosis for lesions of the extrahepatic bile ducts is worse than for lesions of only the intrahepatic ones.
After proctocolectomy, bleeding may develop from varicose veins located around the colostomy.
In non-specific ulcerative colitis, the presence of pericholangitis and sclerosing cholangitis increases the risk of dysplasia and colorectal cancer.
Survival models have been developed to facilitate evaluation of treatment outcomes, to assign patients to groups in clinical trials, and to determine the optimal timing of liver transplantation. The Mayo Clinic model, based on data from five centers that followed 426 patients, takes into account serum bilirubin concentration, histologic stage, patient age, and the presence of splenomegaly. Predicted survival after diagnosis was 78%. Survival was lower in women than in men. The models have little value for individual patients because the disease progresses differently among patients. In addition, the models do not identify patients with cholangiocarcinoma.
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