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Autoimmune hepatitis: Serologic markers

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 characterized by immunologic and autoimmunologic features, generally including the presence of circulating autoantibodies and a high total serum gamma globulin, usually confined to the IgG fraction [1]. Most cases respond to antiinflammatory or immunosuppressive therapy. (See "Autoimmune hepatitis: Treatment".)

The main circulating autoantibodies, although not specific for the disease, are antinuclear antibodies (ANA) and anti-smooth muscle antibodies (ASMA) in type 1 disease and, in type 2 autoimmune hepatitis, anti-liver-kidney microsome-1 antibodies (ALKM-1) and anti-liver cytosol antibody-1 (ALC-1) [2].

The autoantibodies are not believed to be involved in the pathogenesis of autoimmune hepatitis and are not predictive of histologic severity or treatment response [3]. Their main role is to identify patients with autoimmune hepatitis, and thereby point the way toward appropriate therapy and form the basis for a classification (table 1). (See "Autoimmune hepatitis: Disease classification".)

Other circulating autoantibodies in autoimmune hepatitis can also provide help in diagnosing patients without classical autoantibodies, who previously may have been diagnosed as "autoantibody-negative autoimmune hepatitis" or cryptogenic chronic hepatitis. As an example, the presence of autoimmune hepatitis is suggested in a patient with chronic hepatitis who, in the absence of serologic markers of viral hepatitis, has anti-soluble liver/liver pancreas antigen (anti-SLA/LP) or atypical perinuclear antineutrophil cytoplasmic antibodies (atypical pANCA), also referred to as "anti-neutrophil nuclear antibody" (pANNA).

Patients may present with autoimmune hepatitis without circulating autoantibodies, referred to as "autoantibody-negative autoimmune hepatitis." Although infrequent, their course and response to treatment appear to be identical to autoantibody-positive patients [4].

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Literature review current through: Nov 2017. | This topic last updated: Jul 27, 2015.
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