Orphanet: Autoimmune hepatitis

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Autoimmune hepatitis

Disease definition

A rare liver disease characterized by immune-mediated, acute or chronic liver inflammation, clinically presenting as cryptogenic hepatitis, with interface hepatitis on histological examination, elevated serum aminotransferase levels, and hypergammaglobulinemia / elevated immunoglobulin G, in the presence or absence of specific circulating autoantibodies. Patients may be asymptomatic, chronically ill, or present with acute liver failure. Concurrent autoimmune diseases are frequently observed.


Classification level: Disorder
  • Synonym(s):
    • AIH
  • Prevalence: 1-5 / 10 000
  • Inheritance: Not applicable 
  • Age of onset: Adolescent, Elderly, Childhood, Adult
  • ICD-10: K75.4
  • ICD-11: DB96.0
  • OMIM: -
  • UMLS: C0241910
  • MeSH: -
  • GARD: 5871
  • MedDRA: 10003827

Detailed information


Disease review articles

ERN : produced/endorsed by ERN(s)
FSMR : produced/endorsed by FSMR(s)
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