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Liver Immunology pp 249–261Cite as

Sclerosing Cholangitis

Primary and Secondary

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Abstract

Sclerosing cholangitis comprises a spectrum of chronic cholestatic disease of the hepatobiliary system characterized by hepatic inflammation, biliary strictures, and fibrosis. The best studied form is primary sclerosing cholangitis (PSC), which is a slowly progressive disorder eventually resulting in concentric obliterative fibrosis of the bile ducts, biliary cirrhosis, and, in approximately 30% of patients, cholangiocarcinoma. There is a strong association between PSC and inflammatory bowel disease (IBD), with between 75 and 80% of PSC patients of northern European origin having underlying IBD (1,2). Ulcerative colitis is the most common form of IBD associated with PSC, and, interestingly, when considering the pathogenesis of the disease, those PSC patients who have Crohn’s disease almost invariably have disease predominantly affecting the colon.

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Authors and Affiliations

  1. Department of Gastroenterology, John Radcliffe Hospital, Headington, Oxford, UK

    Sue Cullen BSC, MB, BS, MRCP, MD & Roger Chapman BSC (LOND.), MB, BS, FRCP (LOND.), MD (LOND.), MA(OXON)

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  1. Sue Cullen BSC, MB, BS, MRCP, MD
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  2. Roger Chapman BSC (LOND.), MB, BS, FRCP (LOND.), MD (LOND.), MA(OXON)
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Editors and Affiliations

  1. Division of Rheumatology, Allergy and Clinical Immunology, University of California at Davis School of Medicine, Davis, CA

    M. Eric Gershwin MD, FACP

  2. Departments of Medicine and Surgery Baylor Liver Health, Baylor College of Medicine, Houston, TX

    John M. Vierling MD, FACP

  3. Department of Gastroenterology, Hepatology, and Endocrinology, Medical School of Hannover, Hannover, Germany

    Michael P. Manns MD

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© 2007 Humana Press Inc., Totowa, NJ

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Cullen, S., Chapman, R. (2007). Sclerosing Cholangitis. In: Gershwin, M.E., Vierling, J.M., Manns, M.P. (eds) Liver Immunology. Humana Press. https://doi.org/10.1007/978-1-59745-518-3_20

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  • DOI: https://doi.org/10.1007/978-1-59745-518-3_20

  • Publisher Name: Humana Press

  • Print ISBN: 978-1-58829-818-8

  • Online ISBN: 978-1-59745-518-3

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