Anti-mitochondrial antibody positive autoimmune hepatitis triggered by EBV infection in a young girl.

Autoimmune hepatitis (AIH) is rare in children. Two types of childhood autoimmune hepatitis are recognized: AIH type 1 which is characterized by the presence of smooth muscle (SMA) and/or antinuclear (ANA) antibodies; and AIH type 2 which is positive for anti-liver kidney microsomal type 1 (anti-LKM-1) antibody. Anti-mitochondrial antibody (AMA) is considered the hallmark for primary biliary cirrhosis (PBC) that occurs primarily in adult women and is characterized by destruction of the intralobular bile ducts and progression to cirrhosis and liver failure. Antimitochondrial-antibody-positive AIH is extremely rare in children. We report a 13year old Saudi girl with type-1 AIH who had a strongly positive anti-mitochondrial antibody and no evidence of small bile duct disease in the liver biopsy.