Clinical profile of IgG4-related disease in Japan based on the rare disease data registry

Motohisa Yamamoto¹, Masatoshi Kanda², Ichiro Mizushima³, Atsushi Kanno⁴, Takeji Umemura⁵, Tsukasa Ikeura⁶, Yuzo Kodama⁷, Hiroaki Dobashi⁸, Yoshiya Tanaka⁹, Atsushi Masamune¹⁰, Masafumi Moriyama¹¹, Takako Saeki¹², Shoko Matsui¹³, Tomoki Origuchi¹⁴, Yasufumi Masaki¹⁵, Masanori Asada¹⁶, Hisanori Umehara¹⁷, Hiroshi Seno¹⁸, Itaru Naitoh¹⁹, Satoshi Yamamoto²⁰, Eisuke Iwasaki²¹, Kensuke Kubota²², Shiroh Tanoue²³, Takayoshi Nishino²⁴, Hiroto Tsuboi²⁵, Yasushi Matsumoto²⁶, Hiroyuki Isayama²⁷, Hiroshi Goto²⁸, Kenji Notohara²⁹, Kazushige Uchida³⁰, Ken Kawabe³¹, Kazunori Yamada¹⁵, Satomi Kasashima³, Masayuki Takahira³, Yasuharu Sato³², Izumi Kawachi³³, Izumi Yamaguchi¹⁸, Kazuichi Okazaki⁵, Seiji Nakamura¹¹, Fumihiko Matsuda¹⁸, Hideki Ishikawa³⁴, Mitsuhiro Kawano^{3

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56}; Patient Registry Committee, Research Program for Intractable Disease by the Ministry of Health, Labour and Welfare (MHLW), Japan¹⁴

Affiliations

¹ The Institute of Medical Science, The University of Tokyo, Tokyo, Japan.
² Sapporo Medical University, Hokkaido, Japan.
³ Kanazawa University, Ishikawa, Japan.
⁴ Jichi Medical University, Tochigi, Japan.
⁵ Shinshu University, Nagano, Japan.
⁶ Kansai Medical University, Osaka, Japan.
⁷ Kobe University, Hyogo, Japan.
⁸ Kagawa University, Kagawa, Japan.
⁹ University of Occupational and Environmental Health, Fukuoka, Japan.
¹⁰ Tohoku University, Miyagi, Japan.
¹¹ Kyushu University, Fukuoka, Japan.
¹² Nagaoka Red Cross Hospital, Niigata, Japan.
¹³ University of Toyama, Toyama, Japan.
¹⁴ Nagasaki University, Nagasaki, Japan.
¹⁵ Kanazawa Medical University, Ishikawa, Japan.
¹⁶ Japanese Red Cross Osaka Hospital, Osaka, Japan.
¹⁷ Nagahama City Hospital, Shiga, Japan.
¹⁸ Kyoto University, Kyoto, Japan.
¹⁹ Nagoya City University, Aichi, Japan.
²⁰ Fujita Health University Bandane Hospital, Aichi, Japan.
²¹ Keio University, Tokyo, Japan.
²² Yokohama City University, Kanagawa, Japan.
²³ Kagoshima University, Kagoshima, Japan.
²⁴ Tokyo Women's Medical University Yachiyo Medical Center, Chiba, Japan.
²⁵ University of Tsukuba, Ibaraki, Japan.
²⁶ National Hospital Organization Kanazawa Medical Center, Ishikawa, Japan.
²⁷ Juntendo University, Tokyo, Japan.
²⁸ Tokyo Medical University, Tokyo, Japan.
²⁹ Kurashiki Central Hospital, Ohara Health Care Foundation, Okayama, Japan.
³⁰ Kochi University, Kochi, Japan.
³¹ National Hospital Organization Kyushu Medical Center, Fukuoka, Japan.
³² Okayama University, Okayama, Japan.
³³ Niigata University, Niigata, Japan.
³⁴ Kyoto Prefectural University of Medicine, Kyoto, Japan.
³⁵ Drs. Masanari Sugawara (Sapporo Medical University).
³⁶ Shunsuke Tsuge (Kanazawa University).
³⁷ *Other than the co-authors, the members of Patient Registry Committee, Research Program for Intractable Disease by the Ministry of Health, Labour and Welfare (MHLW), Japan are as follows: Kensuke Yokoyama (Jichi Medical University).
³⁸ Eriko Ikeda (Jichi Medical University).
³⁹ Kozue Ando (Jichi Medical University).
⁴⁰ Akira Nakamura (Shinshu University).
⁴¹ Ayaka Takaori (Kansai Medical University).
⁴² Takashi Ito (Kansai Medical University).
⁴³ Koh Nakamaru (Kansai Medical University).
⁴⁴ Noriko Juri (Kobe University).
⁴⁵ Hiromi Shimada (Kagawa University).
⁴⁶ Shingo Nakayamada (University of Occupational and Environmental Health).
⁴⁷ Satoshi Kubo (University of Occupational and Environmental Health).
⁴⁸ Yuya Fujita (University of Occupational and Environmental Health).
⁴⁹ Yoshino Inoue (University of Occupational and Environmental Health).
⁵⁰ Takanori Sano (Tohoku University).
⁵¹ Tetsuya Takikawa (Tohoku University).
⁵² Yuka Miyahara (Kyushu University).
⁵³ Masataka Umeda (Nagasaki University).
⁵⁴ Hajime Yoshifuji (Kyoto University).
⁵⁵ Tomohiro Handa (Kyoto University).
⁵⁶ Masataka Yokode (Kyoto University).

PMID: 39607031
DOI: 10.1080/25785826.2024.2430812

Free article

Clinical profile of IgG4-related disease in Japan based on the rare disease data registry

Motohisa Yamamoto et al. Immunol Med. 2025 Sep.

Free article

. 2025 Sep;48(3):192-202.

doi: 10.1080/25785826.2024.2430812. Epub 2024 Nov 28.

Authors

Affiliations

¹ The Institute of Medical Science, The University of Tokyo, Tokyo, Japan.
² Sapporo Medical University, Hokkaido, Japan.
³ Kanazawa University, Ishikawa, Japan.
⁴ Jichi Medical University, Tochigi, Japan.
⁵ Shinshu University, Nagano, Japan.
⁶ Kansai Medical University, Osaka, Japan.
⁷ Kobe University, Hyogo, Japan.
⁸ Kagawa University, Kagawa, Japan.
⁹ University of Occupational and Environmental Health, Fukuoka, Japan.
¹⁰ Tohoku University, Miyagi, Japan.
¹¹ Kyushu University, Fukuoka, Japan.
¹² Nagaoka Red Cross Hospital, Niigata, Japan.
¹³ University of Toyama, Toyama, Japan.
¹⁴ Nagasaki University, Nagasaki, Japan.
¹⁵ Kanazawa Medical University, Ishikawa, Japan.
¹⁶ Japanese Red Cross Osaka Hospital, Osaka, Japan.
¹⁷ Nagahama City Hospital, Shiga, Japan.
¹⁸ Kyoto University, Kyoto, Japan.
¹⁹ Nagoya City University, Aichi, Japan.
²⁰ Fujita Health University Bandane Hospital, Aichi, Japan.
²¹ Keio University, Tokyo, Japan.
²² Yokohama City University, Kanagawa, Japan.
²³ Kagoshima University, Kagoshima, Japan.
²⁴ Tokyo Women's Medical University Yachiyo Medical Center, Chiba, Japan.
²⁵ University of Tsukuba, Ibaraki, Japan.
²⁶ National Hospital Organization Kanazawa Medical Center, Ishikawa, Japan.
²⁷ Juntendo University, Tokyo, Japan.
²⁸ Tokyo Medical University, Tokyo, Japan.
²⁹ Kurashiki Central Hospital, Ohara Health Care Foundation, Okayama, Japan.
³⁰ Kochi University, Kochi, Japan.
³¹ National Hospital Organization Kyushu Medical Center, Fukuoka, Japan.
³² Okayama University, Okayama, Japan.
³³ Niigata University, Niigata, Japan.
³⁴ Kyoto Prefectural University of Medicine, Kyoto, Japan.
³⁵ Drs. Masanari Sugawara (Sapporo Medical University).
³⁶ Shunsuke Tsuge (Kanazawa University).
³⁷ *Other than the co-authors, the members of Patient Registry Committee, Research Program for Intractable Disease by the Ministry of Health, Labour and Welfare (MHLW), Japan are as follows: Kensuke Yokoyama (Jichi Medical University).
³⁸ Eriko Ikeda (Jichi Medical University).
³⁹ Kozue Ando (Jichi Medical University).
⁴⁰ Akira Nakamura (Shinshu University).
⁴¹ Ayaka Takaori (Kansai Medical University).
⁴² Takashi Ito (Kansai Medical University).
⁴³ Koh Nakamaru (Kansai Medical University).
⁴⁴ Noriko Juri (Kobe University).
⁴⁵ Hiromi Shimada (Kagawa University).
⁴⁶ Shingo Nakayamada (University of Occupational and Environmental Health).
⁴⁷ Satoshi Kubo (University of Occupational and Environmental Health).
⁴⁸ Yuya Fujita (University of Occupational and Environmental Health).
⁴⁹ Yoshino Inoue (University of Occupational and Environmental Health).
⁵⁰ Takanori Sano (Tohoku University).
⁵¹ Tetsuya Takikawa (Tohoku University).
⁵² Yuka Miyahara (Kyushu University).
⁵³ Masataka Umeda (Nagasaki University).
⁵⁴ Hajime Yoshifuji (Kyoto University).
⁵⁵ Tomohiro Handa (Kyoto University).
⁵⁶ Masataka Yokode (Kyoto University).

PMID: 39607031
DOI: 10.1080/25785826.2024.2430812

Abstract

We started a registry for cases of immunoglobulin (Ig)G4-related disease (IgG4-RD) in December 2019 to clarify the clinical profile of IgG4-RD. In this study, clinical information from 854 cases registered by February 16, 2024 was analyzed from multiple perspectives. Diagnosis of IgG4-RD was made in 808 cases, comprising 638 definite, 38 probable, and 132 possible. The mean ± SD age at time of enrollment of the 808 cases was 67.9 ± 11.3 years, with 68.8% being male. The pancreas was the most frequently affected organ (49.8%), followed by the submandibular glands (46.2%) and lacrimal glands (30.6%). This study reconfirmed the pancreas and head-and-neck region as major affected areas in IgG4-RD. Clinically, submandibular adenitis and autoimmune pancreatitis often occur together in the same patient, but no association between the two organs was observed in our analysis. Regarding diagnosis, the comprehensive diagnostic criteria were most commonly used (63.6%). Storiform fibrosis and phlebitis obliterans were detected at different frequencies in different organs. In summary, this registry study identified clinical, imaging, hematologic, and pathologic findings in 808 Japanese patients with IgG4-RD. The frequency of affected organs and their characteristic pathological findings will be particularly useful for future practice.

Keywords: Clinical characteristics; IgG4-related disease; Japan; rare disease platform; registry.

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Clinical profile of IgG4-related disease in Japan based on the rare disease data registry

Affiliations

Clinical profile of IgG4-related disease in Japan based on the rare disease data registry

Authors

Affiliations

Abstract

MeSH terms

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LinkOut - more resources

Full Text Sources

Other Literature Sources

Medical

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