Prognosis

Large cohort studies report median transplant-free survival ranging from 10 to 15 years, reflecting the heterogeneity of the disease. (1)

Prognosis differs by subtype: small-duct PSC generally follows a more indolent course, offering longer transplant-free survival and a substantially lower risk of cholangiocarcinoma than classic large-duct PSC. (2)

Patients usually die from complications related to:

• secondary biliary cirrhosis
• portal hypertension
• cholangitis

About 10% develop cholangiocarcinoma.

Patients who were asymptomatic at the time of diagnosis have a better long-term survival.

There is no relation between the course of the disease and that of accompanying inflammatory bowel disease. PSC often occurs and worsens in patients whose inflammatory bowel disease has become quiescent after colectomy.

Reference

1. Weismüller TJ et al. International PSC Study Group. Patient Age, Sex, and Inflammatory Bowel Disease Phenotype Associate With Course of Primary Sclerosing Cholangitis. Gastroenterology. 2017 Jun;152(8):1975-1984.e8.
2. Björnsson E et al. The natural history of small-duct primary sclerosing cholangitis. Gastroenterology. 2008 Apr;134(4):975-80.