Background: Autoimmune hepatitis (AIH), usually chronic hepatitis, can be revealed by an acute episode of hepatic failure not easily treatable.
Case report: A 4 year-old boy presented with severe acute hepatic failure (AHF) without hypergammaglobulinemia, revealing AIH. A triple immunosuppressive treatment (prednisolone, azathioprine, ciclosporine) was started when clinical and biological signs worsened (prothrombin time 18%, factor V 32%). Liver functions significantly improved within 10 days, although complications due to intensive immunosuppression occurred (Candida septicemia).
Conclusion: AIH must be evocated in each case of AHF. Some observations of AIH treated with ciclosporine are published but protocols of its administration are variable: ciclosporine is used alone or associated with other drugs, in first intention or secondarily, when the classical treatment is contra-indicated or fails. Controlled studies are needed to precise ciclosporine indications in AIH and to propose the best protocol.