Treatment options for primary sclerosing cholangitis

Emmanouil Sinakos, Keith Lindor

Research output: Contribution to journalReview article

14 Scopus citations

Abstract

Primary sclerosing cholangitis is a cholestatic liver disease characterized by inflammation and fibrosis of intra-/extrahepatic bile ducts, leading to multifocal strictures. Primary sclerosing cholangitis exhibits a progressive course resulting in cirrhosis and the need for liver transplantation over a median period of 12 years. The disease is frequently associated with inflammatory bowel disease and carries an increased risk of colorectal cancer and cholangiocarcinoma. Despite extensive research, there is currently no effective medical treatment. Multiple drugs are shown to be ineffective in halting disease progression, including ursodeoxycholic acid, the most widely evaluated drug. High-dose ursodeoxycholic acid (28-30 mg/kg/day) was recently shown to increase the adverse events rate. Endoscopic or radiological dilatation of a 'dominant stricture may lead to symptomatic and biochemical improvement. However, liver transplantation is the only life-prolonging treatment for patients with end-stage disease. Studies with promising drugs, such as antibiotics, antifibrotic agents and bile acid derivatives, are eagerly awaited.

Original languageEnglish (US)
Pages (from-to)473-488
Number of pages16
JournalExpert Review of Gastroenterology and Hepatology
Volume4
Issue number4
DOIs
StatePublished - Aug 1 2010
Externally publishedYes

Keywords

  • cholangiocarcinoma
  • inflammatory bowel disease
  • liver transplantation
  • primary sclerosing cholangitis
  • ursodeoxycholic acid

ASJC Scopus subject areas

  • Hepatology
  • Gastroenterology

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