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Long-term observation of acute-onset autoimmune hepatitis presenting clinically and radiologically as acute hepatitis

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Abstract

Background

There is yet no gold standard for the diagnosis of acute-onset autoimmune hepatitis (A-AIH), especially histologically acute AIH. As a result, long-term observation of A-AIH has been difficult and the nature is not well known. We retrospectively analyzed the clinicopathological features of A-AIH over a long prospective follow-up period.

Methods

Clinical, biochemical, immunological and pathological features of 30 patients (21 female, mean age 55.1 ± 13.1 years) with non-severe A-AIH “without signs of clinical and radiological chronicity” admitted to a community hospital between 2001 and 2015 who were prospectively followed for more than 2 years were analyzed retrospectively.

Results

Liver histology of 45% showed acute and 55% chronic hepatitis. Mean age was older, prothrombin time activity was higher, AIH scores before treatment were lower in histologically acute hepatitis than histologically chronic hepatitis significantly. Liver fibrosis was not coarse, but delicate with severe activity in most patients showing chronic hepatitis defined by our strict criteria. Median (range) follow-up period was 6.9 (2.1–16.2) years. Six (20%) patients experienced episode of relapses. All were alive at the last follow-up point. Corticosteroid was continued at 2.5–5 mg/day until the study end point without serious side effects in most patients. Serial change of alanine aminotransferase levels, immunoglobulin G levels and anti-nuclear antibody titers did not show statistical difference between histologically acute and chronic hepatitis.

Conclusion

Rapid progression of fibrosis could occur in A-AIH. Treatment response and long-term prognosis were good, and not different between patients with histologically acute and chronic hepatitis.

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Abbreviations

AIH:

Autoimmune hepatitis

A-AIH:

Acute-onset autoimmune hepatitis

ALT:

Alanine aminotransferase

IgG:

Immunoglobulin G

ANA:

Anti-nuclear antibody

CS:

Corticosteroid

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Acknowledgements

We are indebted to all our colleagues at our liver units who cared for the patients described herein. We thank Nobuyuki Moriya (Chiba Medical Center) for help in the extraction of old data.

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Authors and Affiliations

  1. Department of Gastroenterology, Graduate School of Medicine, Chiba University, 1-8-1 Inohana, Chuo-ku, Chiba, 260-8670, Japan

    Keiichi Fujiwara, Shin Yasui, Osamu Yokosuka & Naoya Kato

  2. Department of Gastroenterology, Seikeikai Chiba Medical Center, Chiba, 260-0842, Japan

    Yoshihiro Fukuda, Katsushi Seza & Masaya Saito

  3. Division of Pathology, Shonan Fujisawa Tokushukai Hospital, Fujisawa, 251-0041, Japan

    Masayuki Nakano

Authors
  1. Keiichi Fujiwara
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  2. Yoshihiro Fukuda
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  3. Katsushi Seza
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  4. Masaya Saito
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  5. Shin Yasui
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  6. Masayuki Nakano
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  7. Osamu Yokosuka
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  8. Naoya Kato
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Contributions

KF designed and performed the research, analyzed the clinical data and wrote the manuscript. KF, YF, KS, MS, SY, OY and NK contributed to the clinical aspects. KF and MN reviewed the histopathological changes. All authors approved the final version of the manuscript.

Corresponding author

Correspondence to Keiichi Fujiwara.

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Conflict of interest

Keiichi Fujiwara, Yoshihiro Fukuda, Katsushi Seza, Masaya Saito, Shin Yasui, Masayuki Nakano, Osamu Yokosuka and Naoya Kato declare that they have no conflict of interest.

Ethical approval

All procedures followed were in accordance with the ethical standards of the responsible committee on human experimentation and with the Helsinki Declaration of 1975, as revised in 2008.

Informed consent

Informed consent was obtained from all patients for being included in the study.

Electronic supplementary material

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12072_2018_9848_MOESM1_ESM.tiff

Supporting Figure 1. Serial changes of ANA titers of histologically acute (a) and chronic (b) AIH patients after the start of treatment. ANA, anti-nuclear antibody. (TIFF 1521 kb)

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Fujiwara, K., Fukuda, Y., Seza, K. et al. Long-term observation of acute-onset autoimmune hepatitis presenting clinically and radiologically as acute hepatitis. Hepatol Int 12, 191–199 (2018). https://doi.org/10.1007/s12072-018-9848-z

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  • DOI: https://doi.org/10.1007/s12072-018-9848-z

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