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 harmful invaders, such as bacteria. 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 difficult for your liver to carry out essential functions.
Nov. 22, 2014
- Ferri FF. Ferri's Clinical Advisor 2014: 5 Books in 1. Philadelphia, Pa.: Mosby Elsevier; 2014. https://www.clinicalkey.com. Accessed Oct.8, 2014.
- Picco MF (expert opinion). Mayo Clinic, Jacksonville, Fla. Oct. 25, 2014.
- Primary biliary cirrhosis. The National Digestive Diseases Information Clearinghouse. http://digestive.niddk.nih.gov/ddiseases/pubs/primarybiliarycirrhosis/index.aspx. Accessed Oct. 8, 2014.
- Juran BD, et al. Environmental factors in primary biliary cirrhosis. Seminars in Liver Disease. 2014;34:265.
- Lindor, KD, et al. Primary biliary cirrhosis. American Association for the Study of Liver Diseases Practice Guidelines. Hepatology. 2009;50:291.
- Poupon R. Clinical manifestations, diagnosis, and natural history of primary biliary cirrhosis. http://www.uptodate.com/home. Accessed Oct. 8, 2014.
- Boyer TD, et al., eds. Zakim & Boyer's Hepatology: A Textbook of Liver Disease. 6th ed. https://www.clinicalkey.com. Accessed Oct. 8, 2014.
- Poupon R. Overview of the treatment of primary biliary cirrhosis. http://www.uptodate.com/home. Accessed Oct. 8, 2014.
- Flamm S, et al. Liver transplantation in primary biliary cirrhosis. http://www.uptodate.com/home. Accessed Oct. 8, 2014.
- Honda A, et al. Anticholestatic effects of bezafibrate in patients with primary biliary cirrhosis treated with ursodeoxycholic acid. Hepatology. 2013;57:1931.
- AskMayoExpert. What is magnetic resonance elastography (MRE) and what are the indications for an MRE examination? Rochester, Minn.: Mayo Foundation for Medical Education and Research; 2014.
- Singal AK, et al. Model for end-stage liver disease. Journal of Clinical and Experimental Hepatology. 2013;3:50.
- PCBers. http://pbcers.org/. Accessed Oct. 8, 2014.
- Mells GF, et al. Impact of primary biliary cirrhosis on perceived quality of life: The UK-PBC national study. Hepatology. 2013;58:273.
- American Liver Foundation. http://www.liverfoundation.org/support/. Accessed Oct. 21, 2014.
- Venkatesh SK, et al. Magnetic resonance elastography of liver: Clinical applications. Journal of Computer Assisted Tomography. 2013;37:887.
- Imam MH, et al. Pathogenesis and management of pruritus in cholestatic liver disease. Journal of Gastroenterology and Hepatology. 2012;27:1150.
- Bunchorntavakul C, et al. Pruritis in chronic liver disease. Clinics in Liver Disease. 2012;16:331.
- Golden AK. Decision Support System. Mayo Clinic, Rochester, Minn. Oct. 6, 2014.
- Martin P, et al. Evaluation for liver transplantation in adults: 2013 Practice guideline by the American Association for the Study of Liver Diseases and the American Society of Transplantation. Hepatology. 2014;59:1144.
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