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Primary biliary cirrhosis

 

Definition

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 and leads to scarring called cirrhosis.

Alternative Names

PBC

Causes

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

More than half of patients have no symptoms at the time of diagnosis. Symptoms usually come on gradually and may include:

Signs and tests

Tests for liver dysfunction:

Tests for the disease:

Support Groups

Expectations (prognosis)

The outcome can vary. If the condition is not treated, most patients will need a liver transplant to prevent death from this condition. About a quarter of patients who've had the disease for 10 years will experience liver failure. Doctors can now use a statistical model to predict the best time to do the transplant.

Calling your health care provider

Call your health care provider if you have:

Complications

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

Treatments

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 and improves the survival of patients with PBC after they have taken it for 4 years.

Vitamin replacement therapy restores vitamins A, K, and D, which are lost in fatty stools. A calcium supplement should be added to prevent or treat soft, weakened bones (osteomalacia).

Liver transplant before liver failure occurs may be successful.

Prevention

References

Afdhal NH. Diseases of the gall bladder and bile ducts. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier. 2007: chap 159.

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

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


Review Date: 5/4/2010
Reviewed By: David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine; George F. Longstreth, MD, Department of Gastroenterology, Kaiser Permanente Medical Care Program, San Diego, CA. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.
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