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Autoimmune hepatitis

ORPHA:2137· ICD-10 K75.4

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.

Prevalence
1-9 / 100 000
Inheritance
Not applicable
Age of onset
Adolescent, Adult, Childhood, Elderly