Autoimmune hepatitis (AIH) is a progressive necroinflammatory liver disease associated with significant morbidity and mortality. Mainly affecting females, AIH has a varied clinical presentation from minor symptomatology to acute liver failure. The diagnosis should be considered in anyone with abnormal liver function tests. Diagnostic features include biochemical evidence of transaminitis, elevated IgG and positive autoantibodies. Liver biopsy may show interface hepatitis with portal-based plasma cell infiltrates. A clinical and pathological spectrum of disease exists with other autoimmune liver disease in rare cases. AIH responds promptly to immunosuppression therapy, including corticosteroids (prednis(ol)one or budesonide) with azathioprine. Treatment failure can be addressed with several second-line immunosuppressive agents. Liver transplantation remains a successful salvage therapy for acute autoimmune liver failure or treatment failure in chronic AIH complicated by synthetic dysfunction, portal hypertension or hepatocellular carcinoma.
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