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Clinical features of autoimmune hepatitis with acute presentation: a Japanese nationwide survey

  • Original Article—Liver, Pancreas, and Biliary Tract
  • Published:
Journal of Gastroenterology Aims and scope Submit manuscript

Abstract

Background

Autoimmune hepatitis (AIH) is characterized by progressive inflammation and necrosis of hepatocytes and eventually leads to a variety of phenotypes, including acute liver dysfunction, chronic progressive liver disease, and fulminant hepatic failure. Although the precise mechanisms of AIH are unknown, environmental factors may trigger disease onset in genetically predisposed individuals. Patients with the recently established entity of AIH with acute presentation often display atypical clinical features that mimic those of acute hepatitis forms even though AIH is categorized as a chronic liver disease. The aim of this study was to identify the precise clinical features of AIH with acute presentation.

Methods

Eighty-six AIH patients with acute presentation were retrospectively enrolled from facilities across Japan and analyzed for clinical features, histopathological findings, and disease outcomes.

Results

Seventy-five patients were female and 11 were male. Patient age ranged from adolescent to over 80 years old, with a median age of 55 years. Median alanine transaminase (ALT) was 776 U/L and median immunoglobulin G (IgG) was 1671 mg/dL. There were no significant differences between genders in terms of ALT (P = 0.27) or IgG (P = 0.51). The number of patients without and with histopathological fibrosis was 29 and 57, respectively. The patients with fibrosis were significantly older than those without (P = 0.015), but no other differences in clinical or histopathological findings were observed. Moreover, antinuclear antibody (ANA)-positive (defined as × 40, N = 63) and -negative (N = 23) patients showed no significant differences in clinical or histopathological findings or disease outcomes. Twenty-five patients experienced disease relapse and two patients died during the study period. ALP ≥ 500 U/L [odds ratio (OR) 3.20; 95% confidence interval (CI) 1.12–9.10; P < 0.030] and GGT ≥ 200 U/L (OR 2.98; 95% CI 1.01–8.77; P = 0.047) were identified as independent risk factors of disease relapse.

Conclusions

AIH with acute presentation is a newly recognized disease entity for which diagnostic hallmarks, such as ALT, fibrosis, and ANA, are needed. Further investigation is also required on the mechanisms of this disorder. Clinicians should be mindful of disease relapse during patient care.

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Acknowledgements

The authors thank Trevor Ralph for his English editorial assistance.

Funding

This study was conducted and supported by Health Labor Science Research Grants from Research on Measures for Intractable Diseases, the Intractable Hepato-Biliary Diseases Study Group in Japan.

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Authors and Affiliations

  1. Department of Gastroenterology, National Hospital Organization, Shinshu Ueda Medical Center, 1-27-21 Midorigaoka, Ueda, 386-8610, Japan

    Satoru Joshita & Kaname Yoshizawa

  2. Department of Medicine, Division of Gastroenterology and Hepatology, Shinshu University School of Medicine, Matsumoto, Japan

    Satoru Joshita, Kaname Yoshizawa & Takeji Umemura

  3. Research Center for Next Generation Medicine, Shinshu University, Matsumoto, Japan

    Satoru Joshita & Takeji Umemura

  4. Department of Gastroenterology, Fukushima Medical University School of Medicine, Fukushima, Japan

    Hiromasa Ohira & Atsushi Takahashi

  5. Department of Human Pathology, Kanazawa University Graduate School of Medicine, Kanazawa, Japan

    Kenichi Harada & Nguyen Canh Hiep

  6. Department of Pathology Lab. Med., Tokushima University Graduate School, Tokushima, Japan

    Koichi Tsuneyama

  7. Kurume University Research Center for Innovative Cancer Therapy, Kurume, Japan

    Masayoshi Kage

  8. Division of Diagnostic Pathology, Shonan Fujisawa Tokushukai Hospital, Fujisawa, Japan

    Masayuki Nakano

  9. Center for Gastroenterology, Teine Keijinkai Hospital, Sapporo, Japan

    Jong-Hon Kang

  10. Department of Gastroenterology and Hepatology, The Third Hospital of Jikei University School of Medicine, Tokyo, Japan

    Kazuhiko Koike & Mikio Zeniya

  11. Department of Gastroenterology and Hepatology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Science, Okayama, Japan

    Tetsuya Yasunaka & Akinobu Takaki

  12. Division of Gastroenterology, Department of Medicine, Kurume University School of Medicine, Kurume, Japan

    Takuji Torimura

  13. Department of Gastroenterology and Metabology, Ehime University Graduate School of Medicine, Tōon, Japan

    Masanori Abe

  14. Department of Gastroenterology, Graduate School of Medicine, Chiba University, Chiba, Japan

    Osamu Yokosuka

  15. Department of Medicine, Teikyo University School of Medicine, Tokyo, Japan

    Atsushi Tanaka & Hajime Takikawa

Authors
  1. Satoru Joshita
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  2. Kaname Yoshizawa
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  3. Takeji Umemura
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  4. Hiromasa Ohira
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  6. Kenichi Harada
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  8. Koichi Tsuneyama
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  9. Masayoshi Kage
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  10. Masayuki Nakano
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  11. Jong-Hon Kang
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  12. Kazuhiko Koike
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  13. Mikio Zeniya
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  14. Tetsuya Yasunaka
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  15. Akinobu Takaki
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  16. Takuji Torimura
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  17. Masanori Abe
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  18. Osamu Yokosuka
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  19. Atsushi Tanaka
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  20. Hajime Takikawa
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Consortia

The Japan Autoimmune Hepatitis Study Group (JAIHSG)

Corresponding author

Correspondence to Kaname Yoshizawa.

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Conflict of interest

The authors declare that they have nothing to disclose regarding funding from industries or other conflicts of interest with respect to this manuscript.

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Joshita, S., Yoshizawa, K., Umemura, T. et al. Clinical features of autoimmune hepatitis with acute presentation: a Japanese nationwide survey. J Gastroenterol 53, 1079–1088 (2018). https://doi.org/10.1007/s00535-018-1444-4

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  • DOI: https://doi.org/10.1007/s00535-018-1444-4

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