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Background <p>To update the clinic-epidemiological features of autoimmune pancreatitis (AIP) in Japan, we conducted the fifth nationwide survey.</p> Methods <p>We performed a two-stage survey of patients with AIP who were treated at selected hospitals in 2021. The first-stage survey estimated the total number of patients with AIP, and the second-stage survey analyzed detailed clinical characteristics.</p> Results <p>The estimated number of patients with AIP in 2021 was 16,750 (prevalence, 13.3 per 100,000 persons), representing a 25% increase from 2016. The number of newly diagnosed cases was 4210 (annual incidence, 3.4 per 100,000 persons). Clinical data were available for 2833 patients. The male-to-female ratio was 3.32:1, and the mean age in 2021 and the mean age at AIP diagnosis were 71.2 and 66.3&#xa0;years, respectively. At diagnosis, 58.6% of patients were symptomatic, approximately half of whom presented with jaundice. Other organ involvement was observed in 67.8% of patients, most commonly sclerosing cholangitis. Histopathological examination was performed in 84.1% of cases, predominantly using endoscopic ultrasonography-guided tissue acquisition. Initial glucocorticoid therapy was administered to 84.2% of patients, and 93.8% received maintenance therapy. Kaplan–Meier analysis demonstrated a cumulative relapse incidence of 12.5%, 20.7%, 36.0%, 44.0%, 53.3%, and 68.9% at 3, 5, 10, 15, 20, and 25&#xa0;years, respectively. Long-term overall survival was favorable; however, malignancies accounted for 40% of deaths.</p> Conclusions <p>This nationwide survey updates the epidemiological and clinical characteristics of AIP in Japan. Patient numbers continue to rise, and the high cumulative risk of relapse underscores the need for second-line therapies beyond glucocorticoids.</p>

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Nationwide epidemiological survey of autoimmune pancreatitis in Japan in 2021

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  • 发表日期：

    2026年5月19日

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  • Atsushi Masamune,
  • Takanori Sano,
  • Tetsuya Takikawa,
  • Ryotaro Matsumoto,
  • Shin Hamada,
  • Toshiaki Abe,
  • Yuichi Tanaka,
  • Yoshifumi Takeyama,
  • Kazuhiro Kikuta,
  • Takahiro Abe,
  • Makoto Abue,
  • Takumi Akiyama,
  • Toshihiko Arizumi,
  • Shohei Asada,
  • Shunjiro Azuma,
  • Hiroyoshi Doi,
  • Takaaki Eguchi,
  • Nao Fujimori,
  • Akashi Fujita,
  • Akihisa Fukuda,
  • Yoshihisa Fukuda,
  • Hiroyuki Funatyama,
  • Toshifumi Gushima,
  • Sumi Hajime,
  • Yasushi Hamaya,
  • Kiyotaka Hashizume,
  • Ayako Hata,
  • Nobuhiro Hattori,
  • Kazuki Hayashi,
  • Kazunao Hayashi,
  • Koki Hayashi,
  • Nobuhiko Hayashi,
  • Itagaki Hideki,
  • Kawakami Hiroshi,
  • Morihisa Hirota,
  • Endo Hiroyuki,
  • Kazunari Ibusuki,
  • Akihito Ida,
  • Takao Iemoto,
  • Takeshi Iida,
  • Sadaharu Iio,
  • Tsukasa Ikeura,
  • Tsunao Imamura,
  • Tomoko Inagaki,
  • Osamu Inatomi,
  • Kentaro Inoue,
  • Kazunori Ishige,
  • Akio Ishii,
  • Shuichi Ito,
  • Takashi Ito,
  • Tetsuhide Ito,
  • Eisuke Iwasaki,
  • Takuji Iwashita,
  • Keisuke Iwata,
  • Junji Kawaguchi,
  • Naruomi Jinno,
  • Takuo Kado,
  • Seiji Kaino,
  • Masatoshi Kajiwara,
  • Toyoma Kaku,
  • Keiko Kaneko,
  • Atsushi Kanno,
  • Kobashigawa Kasen,
  • Satoshi Kasugai,
  • Shin Kato,
  • Shinya Kawaguchi,
  • Yuki Kawaji,
  • Toru Kawamoto,
  • Nagata Kazuyoshi,
  • Osamu Kido,
  • Yui Kishimoto,
  • Hideki Kitada,
  • Takanori Kitaguchi,
  • Rena Kitano,
  • Masanori Kobayashi,
  • Ikuhiro Kobori,
  • Yuzo Kodama,
  • Kazuhiko Koike,
  • Mitsuhito Koizumi,
  • Shunsuke Komoto,
  • Naoki Konno,
  • Yudai Koya,
  • Kanemitsu Kozue,
  • Atsushi Kubo,
  • Yoshimasa Kubota,
  • Kiyoshi Kume,
  • Hitoshi Kurata,
  • Michio Kuroki,
  • Masaki Kuwatani,
  • Isamu Makino,
  • Toru Maruo,
  • Akinori Maruta,
  • Saburo Matsubara,
  • Kazuyuki Matsumoto,
  • Susumu Matsuo,
  • Ichiro Miyajima,
  • Masaki Miyazawa,
  • Suguru Mizuno,
  • Toshihiro Morita,
  • Shuntaro Mukai,
  • Yoshiyuki Murawaki,
  • Ryoji Nagamura,
  • Kazunari Nakahara,
  • Yousuke Nakai,
  • Akira Nakamura,
  • Kazunori Nakaoka,
  • Hiroshi Nakase,
  • Atsushi Nakazawa,
  • Hyoda Naoko,
  • Yusuke Niina,
  • Tsutomu Nishida,
  • Takayoshi Nishino,
  • Shotaro Noge,
  • Hideharu Ogiyama,
  • Takaya Oguchi,
  • Akihiko Ohata,
  • Tomoyuki Ohno,
  • Masahiro Ohtani,
  • Hiroshi Ohyama,
  • Hiromitsu Oka,
  • Shiro Oka,
  • Toshimasa Okada,
  • Yasuyuki Okada,
  • Hiromasa Okamoto,
  • Kazuichi Okazaki,
  • Fumihiro Okumura,
  • Kosuke Okuwaki,
  • Takao Oyama,
  • Nishio Ryo,
  • Kei Saito,
  • Michihiro Saito,
  • Nozomu Saito,
  • Toshitaka Sakai,
  • Yoshitaka Sakai,
  • Naoki Sasahira,
  • Yutaka Sasaki,
  • Yuichi Sasakura,
  • Masahiro Sato,
  • Satoshi Sato,
  • Yoichiro Sato,
  • Akihiko Satoh,
  • Yugo Sawai,
  • Masanari Sekine,
  • Minoru Shigekawa,
  • Masahiro Shiihara,
  • Yutaka Shimamatsu,
  • Masaaki Shimatani,
  • Takehiro Shimizu,
  • Nobuhiko Shinohara,
  • Hideyuki Shiomi,
  • Yoshio Sogame,
  • Takahiro Suda,
  • Shigeyuki Suenaga,
  • Kaoru Suzuki,
  • Noriaki Suzuki,
  • Rei Suzuki,
  • Yuhei Suzuki,
  • Akinari Tabaru,
  • Kosuke Takagaki,
  • Tadayuki Takagi,
  • Kenichi Takahashi,
  • Kenji Takahashi,
  • Seiichi Takahashi,
  • Dohmen Takahiro,
  • Shinichi Takano,
  • Yuichi Takano,
  • Yusuke Takasaki,
  • Mamoru Takenaka,
  • Eiji Tamoto,
  • Yu Tanaka,
  • Shiroh Tanoue,
  • Shuji Terai,
  • Masao Toki,
  • Yuichi Torisu,
  • Izumi Toshinobu,
  • Takayuki Tsujikawa,
  • Masahiro Tsujimae,
  • Hidehiko Tuda,
  • Kazushige Uchida,
  • Shinya Uemura,
  • Masayuki Ueno,
  • Yoshiyuki Ueno,
  • Jun Unno,
  • Shigeki Wakiyama,
  • Kenta Yamada,
  • Reiko Yamada,
  • Akira Yamamiya,
  • Satoshi Yamamoto,
  • Kohei Yamanouchi,
  • Kentaro Yamao,
  • Hiroaki Yasuda,
  • Ichiro Yasuda,
  • Tatsuji Yogi,
  • Murayama Yoko,
  • Tomoyuki Yokota,
  • Hideo Yoshida,
  • Naoki Yoshida,
  • Hayato Yoshimura,
  • Reiko Yoshioka

摘要

Background

To update the clinic-epidemiological features of autoimmune pancreatitis (AIP) in Japan, we conducted the fifth nationwide survey.

Methods

We performed a two-stage survey of patients with AIP who were treated at selected hospitals in 2021. The first-stage survey estimated the total number of patients with AIP, and the second-stage survey analyzed detailed clinical characteristics.

Results

The estimated number of patients with AIP in 2021 was 16,750 (prevalence, 13.3 per 100,000 persons), representing a 25% increase from 2016. The number of newly diagnosed cases was 4210 (annual incidence, 3.4 per 100,000 persons). Clinical data were available for 2833 patients. The male-to-female ratio was 3.32:1, and the mean age in 2021 and the mean age at AIP diagnosis were 71.2 and 66.3 years, respectively. At diagnosis, 58.6% of patients were symptomatic, approximately half of whom presented with jaundice. Other organ involvement was observed in 67.8% of patients, most commonly sclerosing cholangitis. Histopathological examination was performed in 84.1% of cases, predominantly using endoscopic ultrasonography-guided tissue acquisition. Initial glucocorticoid therapy was administered to 84.2% of patients, and 93.8% received maintenance therapy. Kaplan–Meier analysis demonstrated a cumulative relapse incidence of 12.5%, 20.7%, 36.0%, 44.0%, 53.3%, and 68.9% at 3, 5, 10, 15, 20, and 25 years, respectively. Long-term overall survival was favorable; however, malignancies accounted for 40% of deaths.

Conclusions

This nationwide survey updates the epidemiological and clinical characteristics of AIP in Japan. Patient numbers continue to rise, and the high cumulative risk of relapse underscores the need for second-line therapies beyond glucocorticoids.