Primary biliary cirrhosis, sometimes called PBC, 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 considered an autoimmune disease, in which the body turns against its own cells. Researchers think it is triggered by a combination of genetic and environmental factors. Primary biliary cirrhosis usually develops slowly and medication can slow its progression, especially if treatment begins early.
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.