Primary Sclerosing Cholangitis

Primary sclerosing cholangitis (PSC) causes inflammation and scarring of the bile ducts located inside and outside the liver. The ducts carry bile -- the liquid that helps break down fat in food -- out of the liver. As scarring accumulates, the ducts become blocked causing bile to build up in the liver, which damages liver cells. Eventually, if left untreated, PSC can cause liver failure or hardening of the liver, called cirrhosis.

PSC typically begins between the ages of 30 and 50, occurring most often in men. Although the cause of the condition is unknown, research has shown that it may be an autoimmune disorder, meaning that the immune system mistakes certain body parts and organs as foreign invaders and thus wrongly attacks them. PSC also may be genetically linked to another condition, called ulcerative colitis, which causes inflammation of the colon. It is estimated that 70 percent of PSC patients also suffer from ulcerative colitis. In addition, people with PSC have an increased risk of developing cholangiocarcinoma and cancer of the bile ducts.

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Reviewed by health care specialists at UCSF Medical Center.
Last updated February 14, 2008