Primary Sclerosing Cholangitis

In primary sclerosing cholangitis (PSC), the bile ducts inside and outside the liver become inflamed and scarred. As the scarring increases, the ducts become blocked. The ducts are important because they carry bile out of the liver. Bile is a liquid that helps break down fat in food. If the ducts are blocked, bile builds up in the liver and damages liver cells. Eventually, PSC can cause liver failure.

Researchers do not know what causes PSC. Among the theories under investigation are the possible role of bacteria, viruses, and immune system problems. PSC appears to be associated with ulcerative colitis, a type of inflammatory bowel disease.

The disease usually begins between ages 30 and 60, but the disease can also arise during childhood. PSC is more common in men than women. PSC progresses slowly, so a personcan have the disease for years before symptoms develop. The main symptoms are itching, fatigue, and jaundice, which causes yellowing of the eyes or skin. An infection in the bile ducts can cause chills and fever.

PSC is diagnosed through cholangiography, which involves injecting dye into the bile ducts and taking an x ray. Cholangiography can be performed as an endoscopic procedure (endoscopic retrograde cholangiopancreatography, ERCP), through radiology or surgery, or with magnetic resonance imaging (MRI) scans. Treatment includes medication to relieve itching, antibiotics to treat infections, and vitamin supplements, as people with PSC are often deficient in vitamins A, D, and K. In some cases, surgery to open major blockages in the common bile duct is also necessary. Liver transplantation may be an option if the liver begins to fail.

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For More Information

American Liver Foundation (ALF)
75 Maiden Lane, Suite 603
New York, NY 10038–4810
Phone: 1–800–GO–LIVER (465–4837),
1–888–4HEP–USA (443–7872),
or 212–668–1000
Fax: 212–483–8179
Email: info@liverfoundation.org
Internet: http://www.liverfoundation.org/

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  Additional Information on Primary Sclerosing Cholangitis

The National Digestive Diseases Information Clearinghouse collects resource information on digestive diseases for National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK) Reference Collection. This database provides titles, abstracts, and availability information for health information and health education resources. The NIDDK Reference Collection is a service of the National Institutes of Health.

To provide you with the most up-to-date resources, information specialists at the clearinghouse created an automatic search of the NIDDK Reference Collection. To obtain this information, you may view the results of the automatic search on Primary Sclerosing Cholangitis.

If you wish to perform your own search of the database, you may access and search the NIDDK Reference Collection database online.

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National Digestive Diseases Information Clearinghouse

2 Information Way
Bethesda, MD 20892–3570
Email: http://digestive.niddk.nih.gov/about/contact.htm

The National Digestive Diseases Information Clearinghouse (NDDIC) is a service of the National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK). The NIDDK is part of the National Institutes of Health of the U.S. Department of Health and Human Services. Established in 1980, the Clearinghouse provides information about digestive diseases to people with digestive disorders and to their families, health care professionals, and the public. The NDDIC answers inquiries, develops and distributes publications, and works closely with professional and patient organizations and Government agencies to coordinate resources about digestive diseases.

Publications produced by the Clearinghouse are carefully reviewed by both NIDDK scientists and outside experts.

This publication is not copyrighted. The Clearinghouse encourages users of this publication to duplicate and distribute as many copies as desired.

NIH Publication No. 05–4332
November 2004

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