2021 Jul 18. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2021 Jan–.
Primary sclerosing cholangitis (PSC) is a chronic and progressive cholestatic liver disorder of unknown etiology. Inflammation, fibrosis, and stricturing of intrahepatic and/or extrahepatic biliary ducts characterize PSC. PSC is usually a progressive disorder that leads to complications of cholestasis and liver failure. Median survival from the time of diagnosis to death without liver transplantation is around 10 years.
Established subtypes of PSC are:
Classic: Affects small and large bile ducts
Small-duct: Affects only small bile ducts
Associated with autoimmune hepatitis: Affects small and large bile ducts
PSC is linked to ulcerative colitis and may be complicated by the onset of cholangiocarcinoma. Over the past few decades, the use of endoscopic retrograde cholangiopancreatography (ERCP) has led to more recognition of this disorder. There is no cure for PSC, and the condition is managed with immunosuppressive medications. Some patients end up with a liver transplant, but then they have to deal with an immunosuppressed state and its associated complications.