Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease, characterized by a progressive course of cholestasis with inflammation and fibrosis of the intrahepatic and extrahepatic bile ducts. For unknown reasons men are affected twice as often as women. PSC advances very slowly and patients may remain asymptomatic for years. When symptoms occur, the most common are fatigue, pruritus, weight loss, and jaundice. Ultimately, patients may develop liver failure or cholangiocarcinoma. Approximately 75-90% of patients with primary sclerosing cholangitis (PSC) have inflammatory bowel disease (IBD), most commonly a form of the condition known as ulcerative colitis; however, the course of IBD is not related to PSC.
The mean age at diagnosing primary sclerosing cholangitis is 40 years. Diagnosis is usually based on a combination of clinical features and cholestatic liver function tests with typical cholangiographic findings and confirmed by characteristic histological abnormalities. There is no cure or specific treatment for PSC. Therapy is aimed at treating symptoms and managing complications. Liver transplantation is the only effective therapeutic option for patients with end-stage liver disease from PSC, although high dose ursodeoxycholic acid may have a beneficial effect. In symptomatic patients, the median survival has been estimated to be 12 years from diagnosis. While asymptomatic patients, the majority of whom will develop progressive disease, have a survival rate greater than 70% at 16 years after diagnosis.