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Crohn's Disease and Ulcerative Colitis pp 579–600Cite as

Extraintestinal Manifestations of Inflammatory Bowel Disease: Primary Sclerosing Cholangitis

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Abstract

Primary sclerosing cholangitis (PSC) is a chronic and progressive disease of the biliary tree characterised by concentric, obliterative fibrosis leading to bile duct stricturing and eventually cirrhosis in the majority of cases. The disease course is highly variable between individuals but most patients reach the combined end-point of death or liver transplantation 12–17 years following their diagnosis [1]. One-third of PSC patients will develop cholangiocarcinoma.

Although the underlying aetiopathogenesis of PSC is not yet fully elucidated it is generally accepted to be a condition of immune dysregulation. Unlike most immune mediated conditions, PSC tends to affect men (male: female 2:1), some presenting with fatigue, right upper quadrant abdominal pain, weight loss, pruritus and intermittent jaundice. However, approximately half of the patients are asymptomatic at diagnosis.

Approximately three quarters of the Northern European PSC population have concomitant inflammatory bowel disease (IBD), with the predominant form of IBD being ulcerative colitis (UC). It is likely that PSC/UC represents a distinct UC phenotype. Common features in the PSC/IBD group include: a more benign course, rectal sparing (52% versus 6%), backwash ileitis (51% versus 7%) and an increased prevalence of pouchitis (following colectomy and ileo-anal pouch formation). Because asymptomatic colitis and rectosigmoid sparing are common features in IBD/PSC, all patients with a new diagnosis of PSC should have a full colonoscopy with biopsies.

There is no curative treatment for PSC. Unfortunately, no medical therapy has been proven to improve prognosis in PSC and the use of Ursodeoxycholic acid is controversial. The main cause of death is malignancy, (hepatobiliary and colonic) and liver failure. Liver transplantation is the only therapeutic option with proven benefit for patients with advanced liver disease.

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Authors and Affiliations

  1. Department of Hepatology/Translational Gastroenterology, John Radcliffe Hospital, University of Oxford, Headley Way, Oxford, Oxfordshire, OX3 9DU, UK

    John Halliday MBBS (Hons.), FRACP & Roger W. Chapman MD, FRCP

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  1. John Halliday MBBS (Hons.), FRACP
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  2. Roger W. Chapman MD, FRCP
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Correspondence to Roger W. Chapman MD, FRCP .

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  1. Medical School of the Humboldt Universit, Department of Medicine, Charité Medical Center, Virchow Hospita, Berlin, 13344, Germany

    Daniel C. Baumgart

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Halliday, J., Chapman, R.W. (2012). Extraintestinal Manifestations of Inflammatory Bowel Disease: Primary Sclerosing Cholangitis. In: Baumgart, D. (eds) Crohn's Disease and Ulcerative Colitis. Springer, Boston, MA. https://doi.org/10.1007/978-1-4614-0998-4_47

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