Primary Sclerosing Cholangitis

National Organization for Rare Disorders, Inc.

Important
It is possible that the main title of the report Primary Sclerosing Cholangitis is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

  • Chronic Obliterative Cholangitis
  • Fibrosing Cholangitis
  • Stenosing Cholangitis
  • PSC

Disorder Subdivisions

  • None

General Discussion

Summary
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.
Introduction
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.

Resources

American Autoimmune Related Diseases Association, Inc.
22100 Gratiot Ave.
Eastpointe, MI 48021
Tel: (586)776-3900
Fax: (586)776-3903
Tel: (800)598-4668
Email: aarda@aarda.org
Internet: http://www.aarda.org/

American Liver Foundation
39 Broadway, Suite 2700
New York, NY 10006
USA
Fax: (212)483-8179
Tel: (800)465-4837
Email: http://www.liverfoundation.org/contact/
Internet: http://www.liverfoundation.org

NIH/National Institute of Diabetes, Digestive & Kidney Diseases
Office of Communications & Public Liaison
Bldg 31, Rm 9A06
31 Center Drive, MSC 2560
Bethesda, MD 20892-2560
Tel: (301)496-3583
Email: NDDIC@info.niddk.nih.gov
Internet: http://www2.niddk.nih.gov/

Canadian Liver Foundation
3100 Steeles Avenue East Suite 801
Markham Ontario, L3R 8T3
Canada
Tel: 4164913353
Fax: 9057521540
Tel: 8005635483
Email: clf@liver.ca
Internet: http://www.liver.ca

British Liver Trust
2 Southampton Road
Ringwood, BH24 1HY
United Kingdom
Tel: 01425481320
Fax: 01425481335
Tel: 08006527330
Email: info@britishlivertrust.org.uk
Internet: http://www.britishlivertrust.org.uk

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
TDD: (888)205-3223
Internet: http://rarediseases.info.nih.gov/GARD/

Autoimmune Information Network, Inc.
PO Box 4121
Brick, NJ 08723
Fax: (732)543-7285
Email: autoimmunehelp@aol.com

PSC Partners Seeking a Cure
5237 South Kenton Way
Englewood, CO 80111
Tel: (303)771-5227
Fax: (303)221-0757
Email: contactus@pscpartners.org
Internet: http://www.pscpartners.org

For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders (NORD). A copy of the complete report can be downloaded free from the NORD website for registered users. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational therapies (if available), and references from medical literature. For a full-text version of this topic, 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. NORD recommends that affected individuals seek the advice or counsel of their own personal physicians.

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.

For additional information and assistance about rare disorders, please contact the National Organization for Rare Disorders at P.O. Box 1968, Danbury, CT 06813-1968; phone (203) 744-0100; web site www.rarediseases.org or email orphan@rarediseases.org

Last Updated:  11/20/2013
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