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Oxford Textbook of Medicine (5 ed.)

Edited by David A. Warrell, Timothy M. Cox, John D. Firth

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Latest update

The November 2012 update sees updates to over 70 chapters, focusing on Neurology and Gastroenterology. This update also incorporates a selection of 29 Case Histories taken from related titles in the Oxford Case Histories series, linked to from related chapters. Each case includes several questions followed by detailed answers and discussion to enhance diagnostic and clinical understanding.

Neurology updates include substantial updates to key chapters and new material on a wide range of topics including spinal cord injury, autonomic nervous system disorders, and inherited neurodegenerative diseases. 

Gastroenterology updates include extensive revisions of key chapters on liver failure and acute pancreatitis and new material on a wide range of matters, ranging from the common to the rare: including surgical treatments for colonic diverticular disease, antibody tests for immune disorders, and a revised treatment algorithm for small bowel bacterial overgrowth.

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Publisher:
Oxford University Press
Print Publication Date:
May 2010
Print ISBN-13:
9780199204854
Published to Oxford Medicine:
May 2012
DOI:
10.1093/med/9780199204854.001.1

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Disclaimer

Oxford University Press makes no representation, express or implied, that the drug dosages in this book are correct. Readers must therefore always check the product information and clinical procedures with the most up to date published product information and data sheets provided by the manufacturers and the most recent codes of conduct and safety regulations. The authors and the publishers do not accept responsibility or legal liability for any errors in the text or for the misuse or misapplication of material in this work. Except where otherwise stated, drug dosages and recommendations are for the non-pregnant adult who is not breastfeeding.

Contents

Primary sclerosing cholangitis

Chapter:
Primary sclerosing cholangitis
Author(s):

R.W. Chapman

DOI:
10.1093/med/9780199204854.003.152104_update_001

Update:

The epidemiology of PSC has been updated to reflect further recent studies, including new data on an increased prevalence of Crohn’s disease in PSC than previously recognised. There is further data to support the use of ursodeoxycholic acid in PSC, though not in high doses, and the section on IgG4-associated cholangitis (IAC) has been expanded due to the publication of multiple trials in this area and the increasing recognition of the importance of IAC as a differential diagnosis in PSC. Additonal important references have been added to the “Further Reading” section, including a new Genome Wide Association Study in PSC.

A relevant case history from Oxford Case Histories in Gastroenterology and Hepatology has been added to this chapter.

Updated on 28 November 2012. The previous version of this content can be found here.

Primary sclerosing cholangitis is a chronic cholestatic liver disease caused by diffuse inflammation and fibrosis that can involve the entire biliary tree. The cause is unknown, but presumed to be immune mediated, and there is a very close association with inflammatory bowel disease, particularly ulcerative colitis.

The disorder tends to affect men (male:female 2:1), some presenting with fatigue, intermittent jaundice, weight loss, right upper quadrant pain, and pruritus, but many are asymptomatic at diagnosis, which is made incidentally when a persistently raised serum alkaline phosphatase is discovered, usually in the setting of ulcerative colitis.

Serum biochemical tests usually indicate cholestasis, but diagnosis is based on three criteria: (1) generalized beading and stenosis of the biliary system on cholangiography; (2) absence of choledocholithiasis or a history of bile duct surgery; and (3) exclusion of bile duct cancer, usually by prolonged follow-up.

There is no curative treatment. Pruritus is initially managed with cholestyramine, with second-line treatments including rifampicin and naltrexone. Orthotopic liver transplantation is the only option available for young patients with advanced liver disease.

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