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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
More Names
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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]

References

  1. Primary sclerosing cholangitis. Genetic Home Reference. http://ghr.nlm.nih.gov/condition/primary-sclerosing-cholangitis. Accessed March 4, 2013.
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General Information

  • Genetics Home Reference (GHR) contains information on Primary sclerosing cholangitis. Click on the link to go to GHR and review the information.
  • MedlinePlus, a Web site designed by the National Library of Medicine to help you research your health questions, provides more information about this topic. Click on the link to view this 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.
  • The Merck Manuals Online Medical Library provides information on this condition. Click on the link to view the information.
  • 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.
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs.  Access to this database is free of charge.  Click on the link to read 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.
  • 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.