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Primary biliary cirrhosis

Contents of this page:

Illustrations

Digestive system
Digestive system
Bile pathway
Bile pathway

Alternative Names    Return to top

PBC

Definition    Return to top

Primary biliary cirrhosis is irritation and swelling (inflammation) of the bile ducts of the liver, which blocks the flow of bile. This obstruction damages liver cells.

Causes    Return to top

The cause of inflamed bile ducts in the liver is not known. The disease more commonly affects middle-aged women.

Long-term bile obstruction is believed to lead to liver cirrhosis. The disease may be associated with autoimmune disorders such as:

Symptoms    Return to top

Symptoms usually come on gradually and may include:

Exams and Tests    Return to top

Tests for the disease:

Tests for liver dysfunction:

Treatment    Return to top

Therapy aims to relieve symptoms and prevent complications.

Cholestyramine (or colestipol) may reduce the itching. Ursodeoxycholic acid may improve removal of bile from the bloodstream.

Vitamin replacement therapy restores vitamins A, K, and D, which are lost in fatty stools. A calcium supplement can help prevent soft, weakened bones (osteomalacia).

Liver transplant before liver failure occurs may be successful.

Outlook (Prognosis)    Return to top

The outcome can vary. If the condition is not treated, most patients will need a liver transplant after about 7 years. Doctors can now use statistics to predict the best time to do the transplant.

Possible Complications    Return to top

Progressive cirrhosis can lead to liver failure. Complications can include:

When to Contact a Medical Professional    Return to top

Call your health care provider if you have:

References    Return to top

Silveira MG, Lindor KD. Treatment of primary biliary cirrhosis: therapy with choleretic and immunosuppressive agents. Clin Liver Dis. 2008;12:425-443.

Mayo MJ. Natural history of primary biliary cirrhosis. Clin Liver Dis. 2008;12:277-288.

Update Date: 5/20/2008

Updated by: Christian Stone, MD, Division of Gastroenterology, Washington University in St. Louis School of Medicine, St. Louis, MO. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

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