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Autoimmune hepatitis: Clinical manifestations and diagnosis

Michael A Heneghan, MD, MMedSc, FRCPI
Section Editor
Sanjiv Chopra, MD, MACP
Deputy Editor
Anne C Travis, MD, MSc, FACG, AGAF


Autoimmune hepatitis is a chronic hepatitis that occurs in children and adults of all ages. It is characterized by immunologic and autoimmunologic features, generally including the presence of circulating autoantibodies and high serum globulin concentrations [1]. Since it was first described in the 1950s, the 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 [2].

Type 1 autoimmune hepatitis – Type 1, or classic autoimmune hepatitis, is characterized by circulating antibodies to nuclei (ANA) and/or smooth muscle (ASMA); the latter are thought to be reflective of more specific antiactin antibodies (AAA). AAA is not generally measured in most clinical laboratories, but ASMA with titers of 1:320 or greater generally reflect the presence of AAA. An ELISA technique for detecting antiactin antibodies (IgG anti F-actin) is available in some laboratories and is being used more frequently in commercial laboratories in North America. Other autoantibodies can occur in conjunction with or independent of ANA and ASMA. (See 'Autoantibodies' below and "Autoimmune hepatitis: Serologic markers".)

Type 2 autoimmune hepatitis – Type 2 autoimmune hepatitis is defined by the presence of antibodies to liver/kidney microsomes (ALKM-1) and/or to a liver cytosol antigen (ALC-1) (table 1).

The clinical manifestations of autoimmune hepatitis will be reviewed here. The treatment of this disorder is discussed separately. (See "Autoimmune hepatitis: Treatment".)


Autoimmune hepatitis is seen in all ethnic groups and can occur at any age, though it is often diagnosed in patients in their 40s or 50s [3-5]. It is more common in women than in men (female to male ratio of 3.6 to 1). In studies from Europe, the incidence is 0.9 to 2 per 100,000 population per year, with a prevalence of 11 to 25 per 100,000 population [4-8].


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Literature review current through: Sep 2016. | This topic last updated: Oct 21, 2015.
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