IMR Press / FBE / Volume 4 / Issue 5 / DOI: 10.2741/e490

Frontiers in Bioscience-Elite (FBE) is published by IMR Press from Volume 13 Issue 2 (2021). Previous articles were published by another publisher on a subscription basis, and they are hosted by IMR Press on as a courtesy and upon agreement with Frontiers in Bioscience.


Primary sclerosing cholangitis: etiopathogenesis and clinical management

Show Less
1 Department of Internal Medicine, University of California Davis Health System, 4150 V Street, Sacramento, CA 95817
2 Division of Gastroenterology and Hepatology, University of California Davis Health System, 4150 V Street, Sacramento, CA 95817

*Author to whom correspondence should be addressed.

Academic Editor: Marcelo Gabriel Roma

Front. Biosci. (Elite Ed) 2012, 4(5), 1683–1705;
Published: 1 January 2012
(This article belongs to the Special Issue Experimental and clinical cholestasis)

Primary sclerosing cholangitis (PSC) is a chronic inflammatory liver disease characterized by the destruction of medium to large-sized bile ducts and intense, concentric fibrosis. Complications from PSC include bacterial cholangitis, cirrhosis, and cholangiocarcinoma and a therapy that might alter the natural history of the disease remains lacking. Our understanding of the pathogenesis of PSC also remains rudimentary but several theories exist, suggesting roles for genetic susceptibility, abnormal innate immune responses lymphocyte trafficking, and toxic bile formation. Medical and surgical therapies, short of liver transplantation, have been disappointing. Currently, the management of PSC is aimed largely at the endoscopic treatment of dominant biliary strictures and complications of cholestasis until the disease has progressed to cirrhosis, at which time liver transplantation is indicated. Progress in our basic understanding of PSC is desperately needed in order to rationally design new therapeutic approaches to this disease.

Primary Sclerosing Cholangitis
Inflammatory Bowel Disease
Back to top