Seminars in Immunopathology

, Volume 31, Issue 3, pp 421–435 | Cite as

Autoimmune hepatitis

  • Diego VerganiEmail author
  • Maria Serena Longhi
  • Dimitrios P. Bogdanos
  • Yun Ma
  • Giorgina Mieli-Vergani


Autoimmune hepatitis (AIH) is an inflammatory liver disease affecting mainly females and characterised histologically by interface hepatitis, biochemically by elevated transaminase levels and serologically by the presence of autoantibodies and increased levels of immunoglobulin G. AIH responds to immunosuppressive treatment, which should be instituted as soon as diagnosis is made. Seropositivity for smooth muscle and/or anti-nuclear antibody defines type 1 AIH, while positivity for liver kidney microsomal type 1 antibody defines type 2 AIH. The aetiology of AIH is unknown, though both genetic and environmental factors are involved in its expression. Immune reactions against host liver antigens are believed to be the major mechanism of liver damage.


Autoimmune hepatitis Liver Autoantibody Humoral immunity Cellular immunity Immune-regulation 



autoimmune hepatitis


International Autoimmune Hepatitis Group


immunoglobulin G


anti-nuclear antibodies


anti-smooth muscle antibodies


liver kidney microsomal


anti-soluble liver antigen/liver pancreas antibody


liver cytosol type 1


liver membrane


perinuclear anti-neutrophil cytoplasmic antibodies


cytoplasmic anti-neutrophil cytoplasmic antibodies


perinuclear anti-nuclear neutrophil antibodies


anti-mitochondrial antibody








liver-specific protein


asialoglycoprotein receptor


alcohol dehydrogenase


autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy


UGA tRNA suppressor associated antigenic protein


major histocompatibility complex


human leukocyte antigen


T-cell receptor


cytotoxic T-lymphocyte associated antigen 4


natural killer


Antigen-presenting cell


Tumour necrosis factor alpha






CD4+ CD25+ regulatory T-cells


cytotoxic T lymphocyte


cytochrome P450IID6


cytotoxic T-cells




IL-2 receptor


glucocorticoid inducible tumour necrosis factor receptor


forkhead winged helix transcription factor box P3


interferon gamma


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Copyright information

© Springer-Verlag 2009

Authors and Affiliations

  • Diego Vergani
    • 1
    Email author
  • Maria Serena Longhi
    • 2
  • Dimitrios P. Bogdanos
    • 2
  • Yun Ma
    • 2
  • Giorgina Mieli-Vergani
    • 2
  1. 1.Institute of Liver Studies, Paediatric Liver CentreKing’s College HospitalLondonUK
  2. 2.King’s College London School of MedicineKing’s College HospitalLondonUK

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