Abstract
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.
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Acknowledgments
The authors thank all those who contributed to this nationwide survey. This study was supported by a Health Labour Science Research Grant for Research on Measures for Intractable Disease (Intractable Hepato-Biliary Disease Study Group in Japan) supported by the Ministry of Health, Labour and Welfare of Japan.
Conflict of interest
The authors declare that they have no conflict of interest.
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Participating investigators other than the authors are listed in the Appendix.
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In addition to the authors, the members of the study group who participated and the contributors to this study were as follows: Yutaka Aoyagi (Graduate School of Medical and Dental Science of Niigata University), Yoichi Hiasa (Ehime University Graduate School of Medicine), Norifumi Hibi (Keio University School of Medicine), Kazumasa Hiroishi (Showa University School of Medicine), Yasushi Matsuzaki (Tokyo Medical University Ibaraki Medical Center), Toshio Morizane (International University of Health and Welfare, Shioya Hospital), Hiromasa Ohira (Fukushima Medical University School of Medicine), Yoshiyuki Suzuki (Toranomon Hospital), and Kaname Yoshizawa (Shinshu University School of Medicine).
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Abe, M., Mashiba, T., Zeniya, M. et al. Present status of autoimmune hepatitis in Japan: a nationwide survey. J Gastroenterol 46, 1136–1141 (2011). https://doi.org/10.1007/s00535-011-0421-y
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DOI: https://doi.org/10.1007/s00535-011-0421-y