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Diseases

Genetic and Rare Diseases Information Center (GARD)

Primary biliary cholangitis


Other Names for this Disease
  • PBC
  • Familial primary biliary cirrhosis
  • Primary Biliary Cirrhosis
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Overview

Primary biliary cholangitis (PBC) is a chronic disease that affects the bile ducts within the liver. The bile ducts carry a fluid called bile from the liver to the gallbladder, where it is stored. The bile ducts become inflamed and damaged, which causes bile to build up in the liver. This abnormal buildup destroys liver tissue and in later stages results in cirrhosis. Primary biliary cirrhosis usually occurs between the ages of 40 and 60 and affects women more often than men. The cause of this condition is unknown, but research suggests that it is an autoimmune disease.[1] Ursodiol is the only drug approved by the U.S. Food and Drug Administration for the treatment of primary biliary cirrhosis.[2]

Last updated: 1/20/2016

References

  1. Primary Biliary Cirrhosis. National Digestive Diseases Information Clearinghouse (NDDIC). April 2014; http://digestive.niddk.nih.gov/ddiseases/pubs/primarybiliarycirrhosis/.
  2. Ursodiol Treatments. PBCers. 1/18/2014; http://www.pbcers.org/about-pbc/ursodiol.php.
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Basic Information

  • MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.
  • The Merck Manuals Online Medical Library provides information on this condition for patients and caregivers. 
  • The National Digestive Diseases Information Clearinghouse (NDDIC), part of the National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK), offers information on this condition. Click on the link to view information on this topic.
  • The National Organization for Rare Disorders (NORD) is a federation of more than 130 nonprofit voluntary health organizations serving people with rare disorders. Click on the link to view information on this topic.

In Depth Information

  • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
  • Online Mendelian Inheritance in Man (OMIM) lists the subtypes and associated genes for Primary biliary cholangitis in a table called Phenotypic Series. Each entry in OMIM includes a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine.
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Primary biliary cholangitis. Click on the link to view a sample search on this topic.
Other Names for this Disease
  • PBC
  • Familial primary biliary cirrhosis
  • Primary Biliary Cirrhosis
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.