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Autoimmune hepatitis: Disease classification

Michael A Heneghan, MD, MMedSc, FRCPI
Section Editor
Sanjiv Chopra, MD, MACP
Deputy Editor
Kristen M Robson, MD, MBA, FACG


Autoimmune hepatitis is a chronic hepatitis which can progress to cirrhosis. It is characterized by autoimmune features, hyperglobulinemia, and the presence of circulating autoantibodies. Most cases respond to therapy with immunomodulating drugs. (See "Autoimmune hepatitis: Treatment".)

Since it was first described in the 1950s, this disorder has been known by a variety of terms, including active chronic hepatitis, chronic active hepatitis, chronic aggressive hepatitis, lupoid hepatitis, plasma cell hepatitis, and, most commonly, autoimmune chronic active hepatitis. In 1992, the International Autoimmune Hepatitis Group recommended autoimmune hepatitis as the most appropriate and least redundant term for this disease [1].

Autoimmune hepatitis is characterized by considerable heterogeneity, as illustrated by the variety of clinical features, histologic findings, immunogenetic phenotypes, and circulating autoantibodies (see "Autoimmune hepatitis: Clinical manifestations and diagnosis"). Since the discovery of a distinct form of autoimmune hepatitis seen primarily in children and young adults that is generally characterized by antibodies to a specific cytochrome P450 (IID6) located in liver and kidney microsomes [2], a classification of autoimmune hepatitis based upon circulating autoantibodies has been proposed.

Although extensions of this autoantibody-based classification have been proposed from time to time, none has proven to provide a valid, clinically useful classification for predicting disease course or response to treatment. In addition, there is little evidence to support a role for these antibodies in the pathogenesis of this disorder. (See "Autoimmune hepatitis: Pathogenesis".)

This topic review will discuss the classification of autoimmune hepatitis, while its diagnosis is presented separately (see "Autoimmune hepatitis: Clinical manifestations and diagnosis"). This topic is also discussed in a guideline issued by the American Association for the Study of Liver Diseases (AASLD). This guideline [3] can be accessed through the AASLD website.

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Literature review current through: Nov 2017. | This topic last updated: Feb 24, 2017.
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