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Definition
By Mayo Clinic staff
Primary biliary cirrhosis is a disease in which the bile ducts in your liver are slowly destroyed. Bile, a fluid produced in your liver, plays a role in digesting food and helps rid your body of worn-out red blood cells, cholesterol and toxins. When bile ducts are damaged, as in primary biliary cirrhosis, harmful substances can build up in your liver and sometimes lead to irreversible scarring of liver tissue (cirrhosis).
Primary biliary cirrhosis is thought to be an autoimmune disease in which the body turns against its own cells, but what triggers primary biliary cirrhosis isn't clear.
Primary biliary cirrhosis develops slowly. Medication can slow the progression of the disease, especially if treatment begins early.
- Angulo P, et al. Primary biliary cirrhosis. In: Feldman M, et al. Sleisenger & Fordtran's Gastrointestinal and Liver Disease: Pathophysiology, Diagnosis, Management. 9th ed. Philadelphia, Pa.: Saunders Elsevier; 2010. http://www.mdconsult.com/books/about.do?eid=4-u1.0-B978-1-4160-6189-2..X0001-7--TOP&isbn=978-1-4160-6189-2&about=true&uniqId=229935664-2192. Accessed Oct. 5, 2011.
- Liver biopsy. Alexandria, Va.: American Association for the Study of Liver Diseases. http://www.aasld.org/practiceguidelines/Documents/Bookmarked%20Practice%20Guidelines/PrimaryBillaryCirrhosis7-2009.pdf. Accessed Oct. 5, 2011.
- Primary biliary cirrhosis. National Institute of Diabetes and Digestive and Kidney Diseases. http://digestive.niddk.nih.gov/ddiseases/pubs/primarybiliarycirrhosis/index.aspx. Accessed Oct. 5, 2011.
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