Primary sclerosing cholangitis

Sombat Treeprasertsuk, Keith Lindor

Research output: Chapter in Book/Report/Conference proceedingChapter

Abstract

Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease with a male predominance that usually presents during the fourth decade of life. The disease incidence is 4-13 per million with a prevalence of 100-150 per million. Primary sclerosing cholangitis often leads to cirrhosis and the need for liver transplantation. The pathogeneses is unknown. Most patients have a slowly progressive course without spontaneous resolution. There is no effective therapy. Ursodeoxycholic acid (UDCA) leads to improvement in biochemical abnormalities but not in histology, cholangiographic appearance, or survival. Biliary strictures in PSC patients are treated with endoscopic balloon dilatation or biliary stent placement. Liver transplantation is the best treatment in end-stage PSC with 1-year survival greater than 90% and 5-year survival greater than 83%. The most serious complication of PSC is cholangiocarcinoma, which occurs in up to 10-15% of PSC patients. Further basic and clinical research is needed to improve the care of PSC patients.

Original languageEnglish (US)
Title of host publicationTextbook of Clinical Gastroenterology and Hepatology
Subtitle of host publicationSecond Edition
PublisherWiley-Blackwell
Pages573-579
Number of pages7
ISBN (Print)1405191821, 9781405191821
DOIs
StatePublished - Apr 16 2012

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Keywords

  • Cholangiocarcinoma
  • Inflammatory bowel disease
  • Primary sclerosing cholangitis
  • Small-duct PSC
  • Ursodeoxycholic acid

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Treeprasertsuk, S., & Lindor, K. (2012). Primary sclerosing cholangitis. In Textbook of Clinical Gastroenterology and Hepatology: Second Edition (pp. 573-579). Wiley-Blackwell. https://doi.org/10.1002/9781118321386.ch76