Primary sclerosing cholangitis (PSC) is a cholestatic liver disease characterized by inflammation and fibrosis of the intrahepatic and extrahepatic bile ducts. PSC is typically a slowly progressive disease that is often associated with inflammatory bowel disease (IBD). Many PSC patients are asymptomatic at the time of diagnosis, whereas in others the presence of fatigue or symptoms consistent with biliary obstruction including fever, jaundice, pruritus, and weight loss may be observed. Notably, there is an increased risk for hepatobiliary and colo-rectal malignancies among patients with PSC and IBD.Despite advances in imaging technology and greater awareness of the condition, the median survival free of liver transplantation for patients with large-duct PSC remains stable at 15 years. Much of this is due to the absence of an effective medical therapy.
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