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