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Background: A nationwide survey of autoimmune hepatitis (AIH) was conducted in Japan, and the clinical features of patients with AIH were analyzed., Methods: A total of 1,056 patients were enrolled through questionnaires sent to 153 hospitals and clinics with hepatology specialists., Results: The clinical features of Japanese AIH were as follows: (1) most patients were middle-aged women, with ages peaking in the 60s; (2) serum immunoglobulin G (IgG) levels were high, but more than 30% of the patients had serum IgG levels less than 2,000 mg/dl; the cutoff level of IgG to distinguish between patients with AIH and those with chronic hepatitis resulting from hepatitis virus was approximately 2,000 mg/dl; (3) autoantibodies, particularly the antinuclear antibody, were frequently present; (4) most patients showed histological features of chronic hepatitis or liver cirrhosis, but 10.9% of the patients showed histological features of acute hepatitis; (5) approximately 75% of the patients were treated with corticosteroids and showed a favorable response to treatment, whereas ursodeoxycholic acid was administered to 60% of the patients., Conclusions: The clinical features of patients with AIH in Japan were clarified. The mean age at diagnosis was higher than that in previous studies. In addition, the number of patients with AIH having the histological features of acute hepatitis increased. Diagnosis of AIH does not preclude the presence of acute hepatitis and/or serum IgG levels less than 2,000 mg/dl.

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.

Human leucocyte antigen (HLA)-typing was studied in 82 hepatitis B carriers and 15 cases of autoimmune hepatitis (AIH) to elucidate the genetic factors which may associated with the etio-pathogenesis of chronic hepatitis in Japan. There were two types of HLA phenotypes: HLA A2 was associated with the progression of the disease induced by HBV infection, and HLA A26 and DR5 were related to the retardation of the disease. A family study confirmed the strong association of these HLA phenotypes with the disease induced by HBV infection. In the analysis of AIH, HLA DR4 is a risk factor related to susceptibility of Japanese AIH which shows different clinical manifestations compared to HLA-DR3-positive AIH, dominantly observed in Western countries. These results suggest that genetic background as expressed by HLA, is an important factor in restricting the development of type B hepatitis and AIH, and can explain racial differences of disease susceptibility.

Editorial. Introduces a research paper on the autoimmune reactivity of sera to hepatocyte plasma membrane in type 1 autoimmune hepatitis, published in the March 2000 issue of the `Journal of Gastroenterology.'