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Definition
By Mayo Clinic staffPrimary biliary cirrhosis is a disease in which the bile ducts in your liver are slowly destroyed. Bile, a fluid produced in your liver, is essential for the proper digestion of fats. It also helps rid your body of worn-out red blood cells, cholesterol and toxins. In primary biliary cirrhosis, the destruction of your bile ducts can cause harmful substances to build up in your liver and sometimes lead to irreversible scarring of liver tissue (cirrhosis).
The cause of primary biliary cirrhosis remains unclear. Many experts consider primary biliary cirrhosis an autoimmune disease in which the body turns against its own cells, although it's likely that genetic and environmental factors also play a part. Primary biliary cirrhosis develops slowly. Medication can slow the progression of the disease, especially if treatment begins early.
- Primary biliary cirrhosis. National Institute of Diabetes and Digestive and Kidney Diseases. http://digestive.niddk.nih.gov/ddiseases/pubs/primarybiliarycirrhosis/index.htm. Accessed Aug. 14, 2009.
- Primary biliary cirrhosis. American Liver Foundation. http://www.liverfoundation.org/education/info/pbc/. Accessed Aug. 14, 2009.
- Cirrhosis. The Merck Manuals: The Merck Manual for Healthcare Professionals. http://www.merck.com/mmpe/sec03/ch026/ch026c.html. Accessed Aug. 14, 2009.
- Friedman LS. Liver, biliary tract and pancreas disorders. In: McPhee SJ, et al. Current Medical Diagnosis & Treatment. 48th ed. Los Altos, Calif.: Lange Medical Publications; 2009. http://www.accessmedicine.com/content.aspx?aID=7993. Accessed Aug. 14, 2009.
- Kaplan M, et al. Primary biliary cirrhosis. New England Journal of Medicine. 2005;353:1261.
- Kaplan MM. Clinical manifestations, diagnosis, and natural history of primary biliary cirrhosis. http://www.uptodate.com/home/index.html. Accessed July 30, 2009.
- Kaplan MM. Overview of the treatment of primary biliary cirrhosis. http://www.uptodate.com/home/index.html. Accessed July 30, 2009.
- Hirschfeld GM, et al. Primary biliary cirrhosis associated with HLA, IL12A, and IL12RB2 variants. New England Journal of Medicine. 2009;360:2544.
- Taouli B, et al. Advanced MRI methods for assessment of chronic liver disease. American Journal of Roentgenology. 2009;193:14.
- Lazaridis K, et al. Increased prevalence of antimitochondrial antibodies in first-degree relatives of patients with primary biliary cirrhosis. Hepatology. 2007;46:785.
- Lindor K. Ursodeoxycholic acid for the treatment of primary biliary cirrhosis. New England Journal of Medicine. 2007;357:1254.
- Picco MF (expert opinion). Mayo Clinic, Jacksonville, Fla. Aug. 31, 2009.
- Hay JE. Bone disease in cholestatic liver disease. Gastroenterology. 1995;108:278.
