Autoimmune hepatitis (AIH) is a common cause of acute and chronic hepatitis in childhood. Once the diagnosis is established, treatment with corticosteroid or corticosteroid and azathioprine is indicated. Most children with AIH respond to such therapy and experience remission from active disease. Eliminating drug therapy while maintaining remission is the ultimate goal of therapy. The optimal duration of therapy before drug elimination is unclear. Relapse rate is inversely related to therapy duration before drug withdrawal; thus, discontinuing immunosuppressive treatment is considered only after at least 1 to 2 years of complete remission. When applying a slow and systematic approach, many children with AIH can successfully be weaned off immunosuppression completely. Even patients presenting in acute liver failure may avoid liver transplantation with early medical therapy. In about 10% of patients, treatment fails, requiring alternative therapies and/or liver transplantation as liver disease progresses. Less than 10% of children with autoimmune hepatitis die during 10 years of follow-up.
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