Present status of autoimmune hepatitis in Japan--correlating the characteristics with international criteria in an area with a high rate of HCV infection. Japanese National Study Group of Autoimmune Hepatitis

J Hepatol. 1997 Jun;26(6):1207-12. doi: 10.1016/s0168-8278(97)80453-9.


Background/aims: A nationwide survey of autoimmune hepatitis (AIH) was carried out in Japan.

Methods: Four hundred and ninety-six patients were enrolled by questionnaires sent to 101 hospitals with hepatology specialists.

Results: The clinical features of Japanese AIH were as follows: most patients were middle-aged women; serum autoantibodies, especially antinuclear antibody, were frequently positive, serum IgG level was high, and HLA-DR4 was the major HLA allotype. Liver-kidney microsomal type 1 antibody was positive in nine of 79 patients tested. Eight of these antibody positive patients were also positive for antinuclear antibody and five for anti-smooth muscle antibody. Ninety-two percent of the patients showed piecemeal necrosis and 60% bridging necrosis; plasma cell infiltration in the portal areas was observed in 50% of the patients. Only 12.3% were diagnosed as having liver cirrhosis. A favorable effect of corticosteroid, normalization of serum transaminases, was observed in 89% of 317 patients, who were treated with an initial dose of over 30 mg/day. Sixty-two patients were positive for hepatitis C virus (HCV) markers. In these patients, however, only one patient was liver-kidney microsomal type 1 antibody positive. Corticosteroid was effective in 30 (81%) of 37 HCV-marker-positive patients treated with this agent. Thus the efficacy of corticosteroid did not differ from that in AIH patients without HCV infection (90%). Similarly, interferon treatment was used in 20 patients, all of whom were positive for HCV-RNA, and resulted in 50% efficacy as determined by normalization of the serum transaminase level 6 months after treatment. The International Diagnostic Scoring System for the diagnosis of AIH worked well in these patients, except for HCV-infected individuals, that is, approximately 10% of the total of AIH patients.

Publication types

  • Multicenter Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Age Factors
  • Age of Onset
  • Aged
  • Autoantibodies / blood*
  • Autoimmune Diseases / classification*
  • Autoimmune Diseases / pathology
  • Autoimmune Diseases / virology
  • Child
  • Female
  • HLA-DR4 Antigen / blood
  • Hepatitis / classification
  • Hepatitis / immunology*
  • Hepatitis / pathology
  • Hepatitis C / classification
  • Hepatitis C / immunology*
  • Hepatitis C / pathology
  • Histocompatibility Testing
  • Humans
  • Immunoglobulin G / blood
  • Interferons / therapeutic use
  • Japan
  • Liver / pathology*
  • Liver Function Tests
  • Male
  • Middle Aged
  • Prednisolone / therapeutic use
  • Sex Characteristics
  • Surveys and Questionnaires


  • Autoantibodies
  • HLA-DR4 Antigen
  • Immunoglobulin G
  • Interferons
  • Prednisolone