Primary Sclerosing Cholangitis
You are reading a NORD Rare Disease Report Abstract. NORD’s full collection of reports on over 1200 rare diseases is available to subscribers (click here for details). We are now also offering two full rare disease reports per day to visitors on our Web site.
NORD is very grateful to Keith Lindor, MD, Division of Gastroenterology and Hepatology, Mayo Clinic, for assistance in the preparation of this report.
Synonyms of Primary Sclerosing Cholangitis
- Chronic Obliterative Cholangitis
- Fibrosing Cholangitis
- Stenosing Cholangitis
- No subdivisions found.
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.
Organizations related to Primary Sclerosing Cholangitis
The information in NORD’s Rare Disease Database is for educational purposes only. It should never be used for diagnostic or treatment purposes. If you have questions regarding a medical condition, always seek the advice of your physician or other qualified health professional. NORD’s reports provide a brief overview of rare diseases. For more specific information, we encourage you to contact your personal physician or the agencies listed as “Resources” on this report.
The National Organization for Rare Disorders (NORD) web site, its databases, and the contents thereof are copyrighted by NORD. No part of the NORD web site, databases, or the contents may be copied in any way, including but not limited to the following: electronically downloading, storing in a retrieval system, or redistributing for any commercial purposes without the express written permission of NORD. Permission is hereby granted to print one hard copy of the information on an individual disease for your personal use, provided that such content is in no way modified, and the credit for the source (NORD) and NORD’s copyright notice are included on the printed copy. Any other electronic reproduction or other printed versions is strictly prohibited.
Copyright 1987, 1989, 1997, 2001, 2013
NORD's Rare Disease Information Database is copyrighted and may not be published without the written consent of NORD.