Primary Biliary Cirrhosis

2007-06-02T11:27:00.000-07:00

Primary Biliary Cirrhosis
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Primary biliary cirrhosis is a disease that slowly destroys the liver’s bile ducts. Bile is a substance produced in the liver that helps digest fat in the small intestine and remove toxins from the body. When the ducts are damaged, bile builds up in the liver and damages liver tissue. Biliary cirrhosis can develop over time and may cause the liver to stop working.

The cause of primary biliary cirrhosis is unknown. The disease affects women more often than men and usually occurs between the ages of 30 and 60 years. Some research suggests that the disease may be an autoimmune disorder.

What are the symptoms of primary biliary cirrhosis?
The first and most common symptoms of primary biliary cirrhosis are itchy skin and fatigue. Other symptoms may eventually develop, including

jaundice, which leads to a yellowing of the eyes and skin
fatty deposits under the skin
fluid retention
dry eyes and mouth

In the later stages of the disease, some people develop:

osteoporosis,
arthritis, and
thyroid problems.

How is primary biliary cirrhosis diagnosed?
Primary biliary cirrhosis is diagnosed through laboratory tests, x rays, and in some cases, a liver biopsy to help to determine the extent of disease progression. A liver biopsy uses a thin needle to remove a small sample of liver tissue. The sample is then examined with a microscope.
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How is primary biliary cirrhosis treated?
Initial treatment is usually aimed at relieving symptoms. Vitamin replacement therapy, calcium supplements, and drugs to treat itching are usually prescribed.
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Some patients have also benefitted from ursodeoxycholic acid (Urso 250 and URSO Forte), which is the only drug approved by the U.S. Food and Drug Administration for the treatment of primary biliary cirrhosis. Ursodiol (Actigall) has also helped some patients by increasing bile flow. Neither of these drugs cure the disease, but they can help delay its progression. If the liver becomes severely damaged, a transplant may be necessary.
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For More Information

American Liver Foundation
75 Maiden Lane, Suite 603
New York, NY 10038
Phone:

1–800–GO–LIVER (465–4837),
1–888–4HEPUSA (443–7872), or
212–668–1000
Fax: 212–483–8179
Email: info@liverfoundation.org
Internet: http://www.liverfoundation.org/

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National Digestive Diseases Information Clearinghouse
2 Information Way
Bethesda, MD 20892–3570
Email: nddic@info.niddk.nih.gov

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.

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NIH Publication No. 07–4625
April 2007

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