Show Summary Details
Page of

Autoimmune hepatitis 

Autoimmune hepatitis

Chapter:
Autoimmune hepatitis
Author(s):

H.J.F. Hodgson

DOI:
10.1093/med/9780199204854.003.152102_update_001

Update:

Chapter reviewed, minor updates made, further reading updated.

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

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

PRINTED FROM OXFORD MEDICINE ONLINE (www.oxfordmedicine.com). © Oxford University Press, 2015. All Rights Reserved. Under the terms of the licence agreement, an individual user may print out a PDF of a single chapter of a title in Oxford Medicine Online for personal use (for details see Privacy Policy).

date: 30 March 2017

Autoimmune hepatitis describes chronic inflammation in the liver attributed to immune responses against self-antigens in the liver, typically in the form of a marked portal tract infiltrate containing both plasma cells and T cells. It usually affects women (female:male, 8:1), is often familial, and 60% of patients have other autoimmune diseases (e.g. thyroiditis, type 1 diabetes) in addition.

Presentation may be with anorexia, nausea, hepatic discomfort, and the development of jaundice, but other patients present just with malaise, or with extrahepatic manifestations such as arthralgia, arthritis, or fever. Clinical signs vary greatly: there may be jaundice and tender hepatomegaly; or evidence of fulminant hepatic failure such as encephalopathy; or splenomegaly that indicates that cirrhosis has already developed.

Characteristic laboratory findings include high transaminase levels, marked hyperglobulinaemia (particularly IgG), and circulating autoantibodies (antinuclear antibodies (ANA), titre 1:40 or greater; anti-smooth muscle antibodies (SMA), titre 1:80 or greater; anti-liver–kidney microsomal (LKM-1) antibodies). There are no critical symptoms, signs, biochemical, immunological, or liver biopsy abnormalities that are sufficiently specific to provide diagnostic criteria: diagnosis depends on the constellation of features.

Autoimmune hepatitis tends to progress with the development of hepatic fibrosis and cirrhosis. Severe cases should be treated with an immunosuppressive regimen for 1 to 2 years, typically prednisolone with or without azathioprine in the first instance. The 10-year survival rate is 65% for those presenting with cirrhosis and >95% for those presenting without. Endstage cirrhosis and acute nonresponsive autoimmune hepatitis leading to acute or subacute liver failure are indications for orthotopic liver transplantation.

Access to the complete content on Oxford Medicine Online requires a subscription or purchase. Public users are able to search the site and view the abstracts for each book and chapter without a subscription.

Please subscribe or login to access full text content.

If you have purchased a print title that contains an access token, please see the token for information about how to register your code.

For questions on access or troubleshooting, please check our FAQs, and if you can''t find the answer there, please contact us.