prognosis
Last reviewed 01/2018
The mean survival for all patients is about 7 years, rising to 8-15 years for asymptomatic individuals.
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.