Autoimmune Hepatitis With Severe Hypergammaglobulinemia and Eosinophilia in a Child

Shigeru Makino, Motokazu Nishikado, Hitoshi Awaguni, Ken-ichi Okumura, Jun Shinozuka, Shinsaku Imashuku


We report here a 7-year-old Afghan girl with autoimmune hepatitis (AIH). Because she showed hepatic dysfunction with markedly high serum immunoglobulin G (IgG) (5,234 mg/dL) and eosinophilia (absolute eosinophil counts: 9,547/µL) at onset, we had difficulty in diagnosing this case by differentiating from various possible diseases. After wedge biopsy of the liver which histologically showed chronic hepatitis pattern as well as high titers (1:640) of anti-nuclear antibody with other negative autoantibodies, the patient was eventually diagnosed as AIH type 1 and successfully managed with a combination of prednisolone (PSL), azathioprine and ursodeoxycholic acid (UDCA), followed by a maintenance therapy with PSL, cyclosporine and UDCA.

Int J Clin Pediatr. 2020;9(2):50-54


Autoimmune hepatitis; Hypergammaglobulinemia; Eosinophilia; Autoantibodies; Child

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