title: 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.
abstract: OBJECTIVE
  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.
authors.affiliation: Department of Internal Medicine I, The Jikei University School of Medicine, Minato-ku, Tokyo, Japan.
authors.forename: G
authors.initials: G
authors.lastname: Toda
authors.forename: M
authors.initials: M
authors.lastname: Zeniya
authors.forename: F
authors.initials: F
authors.lastname: Watanabe
authors.forename: M
authors.initials: M
authors.lastname: Imawari
authors.forename: K
authors.initials: K
authors.lastname: Kiyosawa
authors.forename: M
authors.initials: M
authors.lastname: Nishioka
authors.forename: T
authors.initials: T
authors.lastname: Tsuji
authors.forename: M
authors.initials: M
authors.lastname: Omata
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D017668:Age of Onset; D000368:Aged; D001323:Autoantibodies / Q000097:blood*; D001327:Autoimmune Diseases / Q000145:classification* / Q000473:pathology / Q000821:virology; D002648:Child; D005260:Female; D015804:HLA-DR4 Antigen / Q000097:blood; D006505:Hepatitis / Q000145:classification / Q000276:immunology* / Q000473:pathology; D006526:Hepatitis C / Q000145:classification / Q000276:immunology* / Q000473:pathology; D006650:Histocompatibility Testing; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood; D007372:Interferons / Q000627:therapeutic use; D007564:Japan; D008099:Liver / Q000473:pathology*; D008111:Liver Function Tests; D008297:Male; D008875:Middle Aged; D011239:Prednisolone / Q000627:therapeutic use; D012727:Sex Characteristics; D011795:Surveys and Questionnaires
chemical_list: D001323:Autoantibodies; D015804:HLA-DR4 Antigen; D007074:Immunoglobulin G; D007372:Interferons; D011239:Prednisolone