Primary Sclerosing Cholangitis

General Principles

Primary sclerosing cholangitis (PSC) is a cholestatic liver disorder characterized by inflammation, fibrosis, and obliteration of the extrahepatic and/or intrahepatic bile ducts.

  • PSC can be subdivided into small duct and large duct disease. Small duct disease is defined as typical histologic features of PSC with a normal cholangiogram. In large duct disease or classic PSC, typical “beads on a string” strictures of the biliary tree can be detected by cholangiography. Small duct disease carries a more favorable prognosis.1
  • The peak incidence is at about age 40 years. Most patients are middle-aged men, and the male-to-female ratio is 2:1.
  • PSC is frequently associated with IBD (70% of patients have concomitant UC). The clinical course of these conditions is not correlated.
  • PSC increases the risk of colon cancer, in addition to the risk conferred by IBD alone.

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