Immunological Medicine (Dec 2024)
Clinical profile of IgG4-related disease in Japan based on the rare disease data registry
- Motohisa Yamamoto,
- Masatoshi Kanda,
- Ichiro Mizushima,
- Atsushi Kanno,
- Takeji Umemura,
- Tsukasa Ikeura,
- Yuzo Kodama,
- Hiroaki Dobashi,
- Yoshiya Tanaka,
- Atsushi Masamune,
- Masafumi Moriyama,
- Takako Saeki,
- Shoko Matsui,
- Tomoki Origuchi,
- Yasufumi Masaki,
- Masanori Asada,
- Hisanori Umehara,
- Hiroshi Seno,
- Itaru Naitoh,
- Satoshi Yamamoto,
- Eisuke Iwasaki,
- Kensuke Kubota,
- Shiroh Tanoue,
- Takayoshi Nishino,
- Hiroto Tsuboi,
- Yasushi Matsumoto,
- Hiroyuki Isayama,
- Hiroshi Goto,
- Kenji Notohara,
- Kazushige Uchida,
- Ken Kawabe,
- Kazunori Yamada,
- Satomi Kasashima,
- Masayuki Takahira,
- Yasuharu Sato,
- Izumi Kawachi,
- Izumi Yamaguchi,
- Kazuichi Okazaki,
- Seiji Nakamura,
- Fumihiko Matsuda,
- Hideki Ishikawa,
- Mitsuhiro Kawano
Affiliations
- Motohisa Yamamoto
- The Institute of Medical Science, The University of Tokyo, Tokyo, Japan
- Masatoshi Kanda
- Sapporo Medical University, Hokkaido, Japan
- Ichiro Mizushima
- Kanazawa University, Ishikawa, Japan
- Atsushi Kanno
- Jichi Medical University, Tochigi, Japan
- Takeji Umemura
- Shinshu University, Nagano, Japan
- Tsukasa Ikeura
- Kansai Medical University, Osaka, Japan
- Yuzo Kodama
- Kobe University, Hyogo, Japan
- Hiroaki Dobashi
- Kagawa University, Kagawa, Japan
- Yoshiya Tanaka
- University of Occupational and Environmental Health, Fukuoka, Japan
- Atsushi Masamune
- Tohoku University, Miyagi, Japan
- Masafumi Moriyama
- Kyushu University, Fukuoka, Japan
- Takako Saeki
- Nagaoka Red Cross Hospital, Niigata, Japan
- Shoko Matsui
- University of Toyama, Toyama, Japan
- Tomoki Origuchi
- Nagasaki University, Nagasaki, Japan
- Yasufumi Masaki
- Kanazawa Medical University, Ishikawa, Japan
- Masanori Asada
- Japanese Red Cross Osaka Hospital, Osaka, Japan
- Hisanori Umehara
- Nagahama City Hospital, Shiga, Japan
- Hiroshi Seno
- Kyoto University, Kyoto, Japan
- Itaru Naitoh
- Nagoya City University, Aichi, Japan
- Satoshi Yamamoto
- Fujita Health University Bandane Hospital, Aichi, Japan
- Eisuke Iwasaki
- Keio University, Tokyo, Japan
- Kensuke Kubota
- Yokohama City University, Kanagawa, Japan
- Shiroh Tanoue
- Kagoshima University, Kagoshima, Japan
- Takayoshi Nishino
- Tokyo Women’s Medical University Yachiyo Medical Center, Chiba, Japan
- Hiroto Tsuboi
- University of Tsukuba, Ibaraki, Japan
- Yasushi Matsumoto
- National Hospital Organization Kanazawa Medical Center, Ishikawa, Japan
- Hiroyuki Isayama
- Juntendo University, Tokyo, Japan
- Hiroshi Goto
- Tokyo Medical University, Tokyo, Japan
- Kenji Notohara
- Kurashiki Central Hospital, Ohara Health Care Foundation, Okayama, Japan
- Kazushige Uchida
- Kochi University, Kochi, Japan
- Ken Kawabe
- National Hospital Organization Kyushu Medical Center, Fukuoka, Japan
- Kazunori Yamada
- Kanazawa Medical University, Ishikawa, Japan
- Satomi Kasashima
- Kanazawa University, Ishikawa, Japan
- Masayuki Takahira
- Kanazawa University, Ishikawa, Japan
- Yasuharu Sato
- Okayama University, Okayama, Japan
- Izumi Kawachi
- Niigata University, Niigata, Japan
- Izumi Yamaguchi
- Kyoto University, Kyoto, Japan
- Kazuichi Okazaki
- Shinshu University, Nagano, Japan
- Seiji Nakamura
- Kyushu University, Fukuoka, Japan
- Fumihiko Matsuda
- Kyoto University, Kyoto, Japan
- Hideki Ishikawa
- Kyoto Prefectural University of Medicine, Kyoto, Japan
- Mitsuhiro Kawano
- Kanazawa University, Ishikawa, Japan
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
