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Autoimmune Hepatitis: Pathogenesis, Association with Other Syndromes

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Encyclopedia of Medical Immunology

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Synonyms

Autoimmune hepatitis

Definition

Autoimmune hepatitis (AIH) is a rare inflammatory liver disease that is clinically characterized by elevations of transaminases (ALT > AST), immunoglobulins, and certain autoantibodies (AAB) as well as exclusion of other relevant causes of hepatitis (e.g., infectious, toxic, vascular, metabolic). Histological hallmarks are the predominantly lymphocytic, mostly plasma cell-rich portal infiltrates with interface hepatitis, although histology alone is never used as diagnosis. A successful treatment with steroids can strengthen the diagnosis of AIH.

Immunopathogenesis

The presence of liver-specific autoantibodies against cytochromes (UDP-glucuronosyltransferases (UGTs) and formiminotransferase cyclodeaminase (FTCD)), the elevation of immunoglobulins, and the dense lymphocytic portal infiltrates suggested an auto-aggressive cellular immune response. This was further supported by successful therapy with immunosuppressants (Kirk et al. 1980; Manns et...

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Author information

Authors and Affiliations

  1. Department of Gastroenterology, Hepatology and Endocrinology, Hannover Medical School, Carl-Neuberg-Straße 1, 30625, Hannover, Germany

    Richard Taubert, Elmar Jaeckel & Michael P. Manns

Authors
  1. Richard Taubert
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  2. Elmar Jaeckel
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  3. Michael P. Manns
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Corresponding author

Correspondence to Michael P. Manns .

Editor information

Editors and Affiliations

  1. Department of Biochemistry and Molecular Biology, Monash University, Clayton, Victoria, Australia

    Ian R. Mackay

  2. Center for Autoimmune Disease Research, Department of Molecular Microbiology and Immunology, Department of Pathology, Johns Hopkins University, Baltimore, MD, USA

    Noel R. Rose

  3. The Feinstein Institute for Medical Research, Manhasset, NY, USA

    Betty Diamond

  4. The Feinstein Institute for Medical Research, Manhasset, NY, USA

    Anne Davidson

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Taubert, R., Jaeckel, E., Manns, M.P. (2014). Autoimmune Hepatitis: Pathogenesis, Association with Other Syndromes. In: Mackay, I.R., Rose, N.R., Diamond, B., Davidson, A. (eds) Encyclopedia of Medical Immunology. Springer, New York, NY. https://doi.org/10.1007/978-0-387-84828-0_195

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  • DOI: https://doi.org/10.1007/978-0-387-84828-0_195

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  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-0-387-84827-3

  • Online ISBN: 978-0-387-84828-0

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