Clinical profile of IgG4-related disease in Japan based on the rare disease data registry
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.
Authors
- Masatoshi Kanda (ORCID: https://orcid.org/0000-0003-3468-3586)
- Izumi Kawachi (ORCID: https://orcid.org/0000-0002-7140-8500)
- Kazushige Uchida (ORCID: https://orcid.org/0000-0002-3160-3184)
- Hiroshi Seno (ORCID: https://orcid.org/0000-0002-8509-8128)
- Shoko Matsui (ORCID: https://orcid.org/0000-0002-1328-5768)
- Hisanori Umehara (ORCID: https://orcid.org/0000-0001-9833-4647)
- Ken Kawabe (ORCID: https://orcid.org/0000-0001-7235-8560)
- Eisuke Iwasaki (ORCID: https://orcid.org/0000-0001-8010-5117)
- Hiroshi Gotô (ORCID: https://orcid.org/0000-0002-6462-9524)
- Kazuichi Okazaki (ORCID: https://orcid.org/0000-0003-3424-3142)
- Satoshi Yamamoto (ORCID: https://orcid.org/0000-0001-8866-3690)
- Hideki Ishikawa
- Hiroaki Dobashi (ORCID: https://orcid.org/0000-0003-2944-5583)
- Masayuki Takahira (ORCID: https://orcid.org/0000-0003-4266-9602)
- Atsushi Kanno (ORCID: https://orcid.org/0000-0001-6341-5199)
- Itaru Naitoh (ORCID: https://orcid.org/0000-0001-8342-886X)
- Takeji Umemura (ORCID: https://orcid.org/0000-0001-7985-919X)
- Kensuke Kubota (ORCID: https://orcid.org/0000-0001-6767-0410)
- Fumihiko Matsuda (ORCID: https://orcid.org/0000-0003-4557-4553)
- Atsushi Masamune (ORCID: https://orcid.org/0000-0001-7184-7282)
- Hiroto Tsuboi (ORCID: https://orcid.org/0000-0001-9067-0961)
- T. Saeki
- Motohisa Yamamoto (ORCID: https://orcid.org/0000-0003-2742-2484)
- Yasufumi Masaki (ORCID: https://orcid.org/0000-0002-5658-3765)
- Tsukasa Ikeura (ORCID: https://orcid.org/0000-0002-0241-2821)
- Izumi Yamaguchi (ORCID: https://orcid.org/0000-0003-0890-0145)
- Yasuharu Sato (ORCID: https://orcid.org/0000-0001-5234-6861)
- Kenji Notohara (ORCID: https://orcid.org/0000-0001-5618-1312)
- Satomi Kasashima (ORCID: https://orcid.org/0000-0002-9158-9898)
- Yasushi Matsumoto
- M. Sugawara (ORCID: https://orcid.org/0000-0001-7146-533X)
- Shiroh Tanoue (ORCID: https://orcid.org/0000-0003-2625-2698)
- Ichiro Mizushima (ORCID: https://orcid.org/0000-0002-6231-0683)
- Masafumi Moriyama (ORCID: https://orcid.org/0000-0003-2911-2244)
- Masanori Asada (ORCID: https://orcid.org/0000-0002-7478-7649)
- Takayoshi Nishino
- Yuzo Kodama (ORCID: https://orcid.org/0000-0002-1741-5638)
- Yoshiya Tanaka
- Kazunori Yamada
- Mitsuhiro Kawano
- Tomoki Origuchi (ORCID: https://orcid.org/0000-0001-9673-0929)
- Hiroyuki Isayama (ORCID: https://orcid.org/0000-0002-3308-9326)
- Seiji Nakamura
Institutions
- Kanazawa University (JP)
- Kagoshima University (JP)
- Kansai Medical University (JP)
- University of Nagasaki (JP)
- Ministry of Health Labour and Welfare (JP)
- Kyushu University (JP)
- Shinshu University (JP)
- Sapporo Medical University (JP)
- Fujita Health University (JP)
- University of Tsukuba (JP)
- Jichi Medical University (JP)
- Kubota (Japan) (JP)
- Tokyo Medical University (JP)
- Okayama University (JP)
- Kanazawa Medical University (JP)
- Shiga University (JP)
- University of Occupational and Environmental Health Japan (JP)
- Tohoku University (JP)
- Kagawa University (JP)
- Keio University (JP)
- Kyoto University (JP)
- National Kyushu Medical Center (JP)
- Takamatsu University (JP)
- Hanazono University (JP)
- Nagoya City University (JP)
- Juntendo University (JP)
- Kyoto Prefectural University of Medicine (JP)
- Kurashiki Central Hospital (JP)
- Kanazawa Medical Center (JP)
- Yoshino Kogyosho (Japan) (JP)
- Nagaoka Red Cross Hospital (JP)
- Osaka Hospital (JP)
- Fujita Health University Hospital (JP)
- Uji Hospital (JP)
- Kyushu International University (JP)
- Tokyo Women's Medical University (JP)
- National Hospital Organization (JP)
- Juntendo University Hospital (JP)
- Nagahama City Hospital (JP)
- Jichi Medical University Hospital (JP)
- Ōtani University (JP)
- University of Toyama (JP)
- Nagasaki University (JP)
- Tokyo Women's Medical University Yachiyo Medical Center
- Kobe University (JP)
- Niigata University (JP)
- The University of Tokyo (JP)
- Wakayama University (JP)
- Kōchi University (JP)
- Yokohama City University (JP)
Publication Details
- Journal
- Immunological Medicine
- Published
- 2024-11-28
- DOI
- https://doi.org/10.1080/25785826.2024.2430812
- Citations
- 3
- Primary Topic
- IgG4-Related and Inflammatory Diseases
- Type
- article
- Field-Weighted Citation Impact
- 1.41