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Genetic and Rare Diseases Information Center (GARD)

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Primary sclerosing cholangitis


Other Names for this Disease

  • Cholangitis, primary sclerosing
  • Sclerosing cholangitis
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Overview

Primary sclerosing cholangitis (PSC) is characterized by inflammation in the bile ducts (cholangitis) that leads to scarring (sclerosis), narrowing of the ducts, and a buildup of bile in the liver.[1] Early signs and symptoms include extreme tiredness, abdominal pain, and itchiness.[1] As the condition worsens it may cause jaundice, an enlarged spleen, and eventually liver cirrhosis and failure. Other complications may include weight loss, vitamin deficiency, and osteoporosis. Many people with PSC develop other autoimmune conditions such as inflammatory bowel disease, type 1 diabetes, celiac disease, or thyroid disease. PSC is also a risk factor for cancer of the bile ducts (cholangiocarcinoma).[1]
Last updated: 3/4/2013

References

  1. Primary sclerosing cholangitis. Genetics Home Reference. May 2012; http://ghr.nlm.nih.gov/condition/primary-sclerosing-cholangitis. Accessed 3/4/2013.
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Basic Information

  • Genetics Home Reference (GHR) contains information on Primary sclerosing cholangitis. This website is maintained by the National Library of Medicine.
  • 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 Johns Hopkins Medicine Web site has an information page on primary sclerosing cholangitis. Click on the link above to view the information page.
  • The National Cancer Institute provides detailed information on extrahepatic bile duct cancer. Click on the link to view the information page.

In Depth Information

  • Medscape Reference provides information on this topic. Click on the link to view this information. You may need to register to view the medical textbook, but registration is free.
  • 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 sclerosing cholangitis. Click on the link to view a sample search on this topic.
Other Names for this Disease
  • Cholangitis, primary sclerosing
  • Sclerosing cholangitis
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.