Background. Autoimmune hepatitis (AIH) is characterized by increased immunoglobulin G (IgG) levels, the presence of autoantibodies, and various degrees of lymphocyte predominant inflammation and fibrosis histologically. Immunosuppressive therapy induces remission in approximately 80% of those affected. However, liver transplantation is indicated in patients with acute liver failure with encephalopathy at presentation. Liver supporting systems, including plasma exchange (PE) allow bridging patients to transplantation or spontaneous recovery in the setting of liver failure. The role of these systems has not been assessed in children with liver failure of autoimmune etiology.

Case. Herein, we report three cases of AIH with fulminant presentation, with marked symptom resolution with PE as an adjunct therapeutic option to immunosuppressive treatment.

Conclusion. In the setting of AIH, PE may have a special therapeutic role by removing autoantibodies and cytokines, therefore preventing further liver damage and decompensation, and allowing time for recovery.

Keywords: autoimmune hepatitis, children, plasma exchange, treatment