Primary Sclerosing Cholangitis

John E. Eaton, Jayant A. Talwalkar, Keith D. Lindor

Research output: Chapter in Book/Report/Conference proceedingChapter

Abstract

Primary sclerosing cholangitis (PSC) is a chronic cholestatic disorder characterized by inflammation and progressive obliterative fibrosis of the intrahepatic and/or extrahepatic bile ducts which can lead to cirrhosis and complications related to portal hypertension. PSC is strongly associated with inflammatory bowel disease (IBD), particularly ulcerative colitis (UC), as well as an increased risk of hepatobiliary and colorectal malignancy. PSC has several important subtypes, chiefly small duct PSC and PSC-autoimmune hepatitis (PSC-AIH) overlap. The pathophysiology behind PSC is poorly understood, but is likely a complex interaction between genes and the environment. Mounting evidence suggests key immunologic components involved in the development and progression of PSC. Currently, there is no effective medical therapy for PSC and liver transplantation (LT) provides PSC patients with excellent long-term outcomes. Aside from LT in appropriate patients, recognition and treatment of co-morbid conditions and cancer surveillance remain key components to the management of patients with PSC.

Original languageEnglish (US)
Title of host publicationThe Autoimmune Diseases
Subtitle of host publicationFifth Edition
PublisherElsevier Inc.
Pages925-936
Number of pages12
ISBN (Print)9780123849298
DOIs
StatePublished - Dec 1 2013

Keywords

  • Cholangiocarcinoma
  • Cirrhosis
  • Inflammatory bowel disease
  • Primary sclerosing cholangitis
  • Primary sclerosing cholangitis-autoimmune hepatitis overlap syndrome

ASJC Scopus subject areas

  • Immunology and Microbiology(all)

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  • Cite this

    Eaton, J. E., Talwalkar, J. A., & Lindor, K. D. (2013). Primary Sclerosing Cholangitis. In The Autoimmune Diseases: Fifth Edition (pp. 925-936). Elsevier Inc.. https://doi.org/10.1016/B978-0-12-384929-8.00063-0