Autoimmune hepatitis.
Autoimmune hepatitis (AIH) is characterised histologically by interface hepatitis, and serologically by the presence of non-organ and liver specific autoantibodies and increased levels of immunoglobulin G. Its onset is often ill-defined, frequently mimicing acute hepatitis. AIH usually responds to immunosuppressive treatment, which should be instituted as soon as diagnosis is made. Two types of AIH are recognized according to seropositivity for smooth muscle and/or antinuclear antibody (SMA/ANA, type 1 AIH) or liver kidney microsomal type 1 antibody (LKM1, type 2 AIH). There is a female predominance in both. LKM1 positive patients tend to present more acutely, at a younger age and commonly have immunoglobulin A deficiency, while duration of symptoms before diagnosis, clinical signs, family history of autoimmunity, presence of associated autoimmune disorders, response to treatment and long-term prognosis are similar in the 2 groups. Susceptibility to AIH type 1 is conferred by possession of HLA DR3 and DR4, while to AIH type 2 by possession of HLA DR7. Liver damage is likely to derive from an immune reaction to liver cell antigens, possibly triggered by a mechanism of molecular mimicry, where immune responses to external pathogens, e.g. viruses, become directed towards structurally similar self-components. In AIH this process would be perpetuated by impairment in immune regulation.
Vergani
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D
Institute of Liver Studies, King's College Hospital, London, UK. diego.vergani@kcl.ac.uk
Mieli-Vergani
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G
D000367:Age Factors; D000893:Anti-Inflammatory Agents / Q000008:administration & dosage / Q000627:therapeutic use; D001323:Autoantibodies / Q000032:analysis; D001706:Biopsy; D002648:Child; D016572:Cyclosporine / Q000008:administration & dosage / Q000627:therapeutic use; D005260:Female; D005455:Fluorescent Antibody Technique; D019693:Hepatitis, Autoimmune / Q000145:classification / Q000175:diagnosis / Q000276:immunology / Q000473:pathology / Q000503:physiopathology / Q000628:therapy; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage / Q000627:therapeutic use; D008099:Liver / Q000473:pathology; D008111:Liver Function Tests; D008297:Male; D009173:Mycophenolic Acid / Q000008:administration & dosage / Q000031:analogs & derivatives* / Q000627:therapeutic use; D011239:Prednisolone / Q000008:administration & dosage / Q000627:therapeutic use; D011379:Prognosis; D013997:Time Factors
D000893:Anti-Inflammatory Agents; D001323:Autoantibodies; D007166:Immunosuppressive Agents; D016572:Cyclosporine; D011239:Prednisolone; D009173:Mycophenolic Acid