Primary Sclerosing Cholangitis
National Organization for Rare Disorders, Inc.
It is possible that the main title of the report Primary Sclerosing Cholangitis is not the name you expected.
Primary sclerosing cholangitis (PSC) is a rare progressive disorder characterized by inflammation, thickening, and abnormal formation of fibrous tissue (fibrosis) within the passages that carry bile from the liver (bile ducts). Both the bile ducts within the liver (intrahepatic) and outside the liver (extrahepatic) are affected. This often results in the obstruction or interruption of bile flow from the liver (cholestasis). Symptoms associated with PSC include fatigue and itching (pruritus), followed by yellowing of the skin, mucous membranes, and whites of the eyes (jaundice). Affected individuals may also have dark urine, light-colored stools, abdominal pain, and/or nausea. In some cases, the liver may also become abnormally enlarged (hepatomegaly). Scarring of the liver (cirrhosis) eventually develops and many individuals will ultimately require a liver transplant. According to the medical literature, approximately 60 to 80 percent of individuals with PSC also have inflammatory bowel disease (IBD), most often ulcerative colitis. The relationship between these disorders and the exact cause of PSC are not fully understood.
PSC is a complex disorder and the cause (etiology) and underlying manner the disease develops (pathogenesis) are not fully understood. PSC was first described in the medical literature in 1867. Some researchers believe that PSC represents a group of disorders or a disorder with several distinct subtypes (e.g. PSC with IBD or without IBD). It is likely that PSC may have different underlying causes in different individuals. PSC is a rapidly evolving disease concept and information about PSC is constantly changing and emerging as researchers work to better understand this disorder.
American Autoimmune & Related Diseases
- 22100 Gratiot Ave.
- Eastpointe, MI 48021
- Tel: (586)776-3900
- Fax: (586)776-3903
- Tel: (800)598-4668
- Email: firstname.lastname@example.org
- Website: http://www.aarda.org/
American Liver Foundation
- 39 Broadway, Suite 2700
- New York, NY 10006
- Fax: (212)483-8179
- Tel: (800)465-4837
- Email: http://www.liverfoundation.org/contact/
- Website: http://www.liverfoundation.org
British Liver Trust
- 2 Southampton Road
- Ringwood, BH24 1HY
- United Kingdom
- Tel: 1425481320
- Fax: 1425481335
- Tel: 8006527330
- Email: email@example.com
- Website: http://www.britishlivertrust.org.uk
Canadian Liver Foundation
- 3100 Steeles Avenue East Suite 801
- Markham Ontario, L3R 8T3
- Tel: 4164913353
- Fax: 9057521540
- Tel: 8005635483
- Email: firstname.lastname@example.org
- Website: http://www.liver.ca
Genetic and Rare Diseases (GARD) Information Center
- PO Box 8126
- Gaithersburg, MD 20898-8126
- Tel: (301)251-4925
- Fax: (301)251-4911
- Tel: (888)205-2311
- Website: http://rarediseases.info.nih.gov/GARD/
NIH/National Institute of Diabetes, Digestive & Kidney Diseases
- Office of Communications & Public Liaison
- Bldg 31, Rm 9A06
- Bethesda, MD 20892-2560
- Tel: (301)496-3583
- Email: NDDIC@info.niddk.nih.gov
- Website: http://www2.niddk.nih.gov/
PSC Partners Seeking a Cure
- 5237 South Kenton Way
- Englewood, CO 80111
- Tel: (303)771-5227
- Fax: (303)221-0757
- Email: email@example.com
- Website: http://www.pscpartners.org
For a Complete Report
This is an abstract of a report from the National Organization for Rare Disorders (NORD). For a full-text version of this report, go to www.rarediseases.org and click on Rare Disease Database under "Rare Disease Information".
The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only.
It is possible that the title of this topic is not the name you selected. Please check the Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report.
This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.
Last Updated: 11/20/2013
Copyright 2013 National Organization for Rare Disorders, Inc.
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