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Alternative Names Return to topPBC
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.