CausesBy Mayo Clinic staff
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|Bile duct damage|
It's not clear what causes primary biliary cirrhosis. Many experts consider primary biliary cirrhosis an autoimmune disease in which the body turns against its own cells.
How primary biliary cirrhosis develops
The inflammation of primary biliary cirrhosis begins when T lymphocytes (T cells) start accumulating in your liver. T cells are white blood cells that are part of your immune system response. Normally, T cells recognize and help defend against bacteria and fungi. But in primary biliary cirrhosis, the T cells invade and destroy the cells lining the small bile ducts in your liver.
Inflammation in the smallest ducts spreads, in time, and destroys nearby liver cells. As these cells are destroyed, they're replaced by scar tissue (fibrosis) that can contribute to cirrhosis. Cirrhosis is scarring of liver tissue that makes it less and less possible for your liver to carry out essential functions.
- 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.