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Nationwide epidemiological survey of autoimmune pancreatitis in Japan in 2016

  • Original Article—Liver, Pancreas, and Biliary Tract
  • Published:
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Abstract

Background

To further clarify the clinico-epidemiological features of autoimmune pancreatitis (AIP) in Japan, we conducted the fourth nationwide epidemiological survey.

Methods

This study consisted of two stage surveys; the number of AIP patients was estimated by the first survey and their clinical features were assessed by the second survey. We surveyed the AIP patients who had visited hospitals in 2016.

Results

The estimated number of AIP patients in 2016 was 13,436, with an overall prevalence rate of 10.1 per 100,000 persons. The estimated number of newly diagnosed patients was 3984, with an annual incidence rate of 3.1 per 100,000 persons. Compared to the 2011 survey, both numbers more than doubled. We obtained detailed clinical information of 1474 AIP patients. The male-to-female sex ratio was 2.94, the mean age was 68.1, and mean age at diagnosis was 64.8. At diagnosis, 63% patients were symptomatic and nearly half of them presented jaundice. Pancreatic cysts were found in 9% of the patients and calcifications in 6%. Histopathological examination was performed in 64%, mainly by endoscopic ultrasonography-guided fine needle aspiration. Extra-pancreatic lesions were detected in 60% of the patients. Eighty-four % patients received the initial steroid therapy, and 85% received maintenance steroid therapy. Kaplan–Meier analysis revealed that the relapsed survival was 14% at 3 years, 25% at 5 years, 40% at 10 years, and 50% at 15 years. Mortality was favorable, but pancreatic cancer accounted for death in one quarter of fatal cases.

Conclusion

We clarified the current status of AIP in Japan.

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Abbreviations

AIP:

Autoimmune pancreatitis

EUS-FNA:

Endoscopic ultrasonography-guided fine needle aspiration

ICDC:

International consensus diagnostic criteria

Ig:

Immunoglobulin

IgG4-RD:

IgG4-related disease

JPS:

Japan Pancreas Society

MPD:

Main pancreatic duct

PSL:

Prednisolone

SD:

Standard deviation

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Acknowledgements

The authors are grateful to the doctors who replied to the questionnaires.

Collaborators: Atsushi Kanno, Takanori Sano, Kazushige Uchida, Tsukasa Ikeura, Nao Fujimori, Yousuke Nakai, Terumi Kamisawa, Kensuke Kubota, Masayo Motoya, Kyoko Shimizu, Tomoyuki Ushijima, Mitsuharu Fukasawa, Itaru Naitoh, Masayuki Ueno, Kosuke Okuwaki, Norimitsu Uza, Masanori Asada, Tsuyoshi Mukai, Yasushi Kudo, Kota Uetsuki, Akira Mitoro, Hiroyuki Watanabe, Shuji Terai, Kazunao Hayashi, Yoshiki Imamura, Shin Haba, Kazuo Hara, Toshio Fujisawa, Eisuke Iwasaki, Fumihiro Okumura, Yuki Kawaji, Masayuki Kitano, Takayoshi Nishino, Masatsugu Nagahama, Atsuyuki Hirano, Yukio Aruga, Satoshi Yamamoto, Kazuo Inui, Yoshimasa Kubota, Kuniyuki Takahashi, Junichi Sakagami, Takashi Kobayashi, Takashi Miraki, Takayuki Watanabe, Hiroko Sato, Yasuto Takeda, Masahiro Satoh, Kasen Kobashigawa, Mitsuru Chiba, Tetsuo Tamura, Naoya Sawada, Kazuhiro Mizukami, Masanari Sekine, Jun Unno, Tetsuya Ishizawa, Hiroyuki Funayama, Katsuko Hatayama, Hiroyuki Miyakawa, Toshitaka Sakai, Masaki Kuwatani, Masao Toki, Tatsuya Toyokawa, Shinichi Hashimoto, Takao Itoi, Yuji Iwashita, Ai Sato, Hironari Kato, Hajime Takatori, Yusuke Iizawa, Takuji Iwashita, Hiromu Kondo, Yuichirou Sato, Minoru Tanabe, Toshiro Ogura, Katsutoshi Yamamoto, Tomohiro Masaka, Morimichi Setsuda, Tatsushi Sano, Saburo Matsubara, Katsuya Kitamura, Keisuke Iwata, Nakao Shirahata, Hideki Hagiwara, Motoyuki Yoshida, Tomoyuki Yokota, Hiroaki Shigoka, Naoya Kaneko, Makoto Abue, Nanae Tsuruoka.

Funding

This work was supported in part by the Japan Pancreas Society, Research Committee to establish diagnostic criteria and treatment for systemic IgG4-related sclerosing disease, and Division of Gastroenterology, Tohoku University Graduate School of Medicine.

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Correspondence to Atsushi Masamune.

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Supplementary file1 (DOCX 27 kb)

535_2019_1658_MOESM2_ESM.pdf

Supplementary Figure 1. Distribution of age at diagnosis stratified by gender. Supplementary Figure 2. Distribution of year of diagnosis in the reported cases. Distribution of year of diagnosis (in 20XX) is shown. Among the reported cases, the year of diagnosis was not described in 6 cases. Supplementary Figure 3. Timing of diagnosis of malignant diseases in AIP patients. The information was available in 261 malignant diseases. Fifty-one (19.6%) malignant diseases were detected simultaneously with the AIP diagnosis.(PDF 112 kb)

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Masamune, A., Kikuta, K., Hamada, S. et al. Nationwide epidemiological survey of autoimmune pancreatitis in Japan in 2016. J Gastroenterol 55, 462–470 (2020). https://doi.org/10.1007/s00535-019-01658-7

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  • DOI: https://doi.org/10.1007/s00535-019-01658-7

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