Primary Sclerosing Cholangitis

Tuesday, January 28, 2003

Primary sclerosing cholangitis (PSC) is a liver disease in which the bile ducts become inflamed and scarred, constricting the ducts and preventing bile from flowing out of the liver. As a result, bile that normally would be carried out of the liver accumulates in the liver, damaging the cells.

No exact cause of PSC has been identified and there is no evidence to suggest that the disease is inherited. Because no symptoms appear for many years, the patient is often unaware of his or her disease. PSC is usually diagnosed during a liver function test, when liver abnormalities show up.

The disease usually presents between ages 30 and 60 and is more common in men than in women. Major symptoms are itching, fatigue and jaundice.

There is no known treatment for PSC. Certain medical procedures can be used to treat symptoms at various stages of the disease, such as drugs, or endoscopic procedures to open blockages in the bile ducts.

When the patient with PSC is ill for a long time and then develops end stage liver disease, liver transplantation is indicated as an effective treatment. Liver transplantation is successful in 80 to 90 percent of adult patients and a good quality of life can be enjoyed.

In the case of Mayo Clinic patient Robert Espinoza, 64, there was concern that his liver may have developed cancer because of the PSC. He was monitored very carefully over a period of months to make sure cancer did not develop, which could have compromised his chances for a liver transplant. Mr. Espinoza was successfully transplanted on Jan. 22, 2003.

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