Validation of the Diagnostic Criteria for IgG4-Related Periaortitis/Periarteritis and Retroperitoneal Fibrosis (IgG4PA/RPF) 2018, and Proposal of a Revised 2023 Version for IgG4-Related Cardiovascular/Retroperitoneal Disease

Ichiro Mizushima; Noriyasu Morikage; Eisaku Ito; Fuminori Kasashima; Yasushi Matsumoto; Naoki Sawa; Hajime Yoshifuji; Takako Saeki; Yukako Shintani-Domoto; Shogo Shimada; Toshio Takayama; Eisuke Amiya; Makiko Ozawa; Masaaki Takahashi; Yasunari Fujinaga; Takahiro Katsumata; Yukio Obitsu; Atsushi Izawa; Hiroyuki Kanno; Noriko Oyama-Manabe; Nobukazu Ishizaka; Tasuku Nagasawa; Hiroki Takahashi; Takao Ohki; Mitsuhiro Kawano; Satomi Kasashima; Joint Working Group from the Japanese Circulation Society and the Ministry of Health, Labour and Welfare Study Group

doi:10.1253/circj.CJ-24-0026

Ichiro Mizushima , Noriyasu Morikage, Eisaku Ito, Fuminori Kasashima, Yasushi Matsumoto, Naoki Sawa, Hajime Yoshifuji, Takako Saeki, Yukako Shintani-Domoto, Shogo Shimada, Toshio Takayama, Eisuke Amiya, Makiko Ozawa, Masaaki Takahashi, Yasunari Fujinaga, Takahiro Katsumata, Yukio Obitsu, Atsushi Izawa, Hiroyuki Kanno, Noriko Oyama-Manabe, Nobukazu Ishizaka, Tasuku Nagasawa, Hiroki Takahashi, Takao Ohki, Mitsuhiro Kawano, Satomi Kasashima, Joint Working Group from the Japanese Circulation Society and the Ministry of Health, Labour and Welfare Study Group

Author information

Ichiro Mizushima Corresponding author
Department of Nephrology and Rheumatology, Kanazawa University Hospital [Japan]
Noriyasu Morikage
Division of Vascular Surgery, Department of Surgery and Clinical Science, Yamaguchi University Graduate School of Medicine [Japan]
Eisaku Ito
Division of Vascular Surgery, Department of Surgery, The Jikei University School of Medicine [Japan]
Fuminori Kasashima
Department of Cardiovascular Surgery, Kanazawa Medical Center [Japan]
Yasushi Matsumoto
Department of Cardiovascular Surgery, Kanazawa Medical Center [Japan]
Naoki Sawa
Department of Nephrology and Rheumatology, Toranomon Hospital [Japan]
Hajime Yoshifuji
Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University [Japan]
Takako Saeki
Department of Internal Medicine, Nagaoka Red Cross Hospital [Japan]
Yukako Shintani-Domoto
Department of Diagnostic Pathology, Nippon Medical School Hospital [Japan]
Shogo Shimada
Department of Cardiac Surgery, The University of Tokyo [Japan]
Toshio Takayama
Division of Vascular Surgery, Department of Surgery, Graduate School of Medicine, The University of Tokyo [Japan]
Eisuke Amiya
Department of Cardiovascular Medicine, Graduate School of Medicine, The University of Tokyo [Japan]
Makiko Ozawa
Department of Internal Medicine, Matsumoto Dental University Hospital [Japan]
Masaaki Takahashi
Department of Radiology, Shinshu University School of Medicine [Japan]
Yasunari Fujinaga
Department of Radiology, Shinshu University School of Medicine [Japan]
Takahiro Katsumata
Department of Thoracic and Cardiovascular Surgery, Osaka Medical and Pharmaceutical University [Japan]
Yukio Obitsu
Department of Vascular Surgery, International University of Health and Welfare Mita Hospital [Japan]
Atsushi Izawa
School of Health Sciences, Shinshu University [Japan]
Hiroyuki Kanno
Department of Pathology, Shinshu University School of Medicine [Japan]
Noriko Oyama-Manabe
Department of Radiology, Jichi Medical University Saitama Medical Center [Japan]
Nobukazu Ishizaka
Health Check-up Center, Doai Memorial Hospital [Japan]
Tasuku Nagasawa
Division of Nephrology, Endocrinology, and Vascular Medicine, Tohoku University Hospital [Japan]
Hiroki Takahashi
Department of Rheumatology and Clinical Immunology, Sapporo Medical University School of Medicine [Japan]
Takao Ohki
Division of Vascular Surgery, Department of Surgery, The Jikei University School of Medicine [Japan]
Mitsuhiro Kawano
Department of Nephrology and Rheumatology, Kanazawa University Hospital [Japan]
Satomi Kasashima
Department of Clinical Laboratory Science, Graduate School of Health Science, Kanazawa University [Japan]
Joint Working Group from the Japanese Circulation Society and the Ministry of Health, Labour and Welfare Study Group

Keywords: IgG4-related disease, Organ-specific diagnostic criteria, Periaortitis/periarteritis, Retroperitoneal fibrosis, Revision

JOURNAL OPEN ACCESS FULL-TEXT HTML Advance online publication

Article ID: CJ-24-0026

DOI https://doi.org/10.1253/circj.CJ-24-0026

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Abstract

Background: In 2018, diagnostic criteria were introduced for IgG4-related periaortitis/periarteritis and retroperitoneal fibrosis (PA/RPF). This study assessed the existing criteria and formulated an improved version.

Methods and Results: Between August 2022 and January 2023, we retrospectively analyzed 110 Japanese patients diagnosed with IgG4-related disease (IgG4-RD) involving cardiovascular and/or retroperitoneal manifestations, along with 73 non-IgG4-RD patients (“mimickers”) identified by experts. Patients were stratified into derivation (n=88) and validation (n=95) groups. Classification as IgG4-RD or non-IgG4-RD was based on the 2018 diagnostic criteria and various revised versions. Sensitivity and specificity were calculated using experts’ diagnosis as the gold standard for the diagnosis of true IgG4-RD and mimickers. In the derivation group, the 2018 criteria showed 58.5% sensitivity and 100% specificity. The revised version, incorporating “radiologic findings of pericarditis”, “eosinophilic infiltration or lymphoid follicles”, and “probable diagnosis of extra-PA/-RPF lesions”, improved sensitivity to 69.8% while maintaining 100% specificity. In the validation group, the original and revised criteria had sensitivities of 68.4% and 77.2%, respectively, and specificities of 97.4% and 94.7%, respectively.

Conclusions: Proposed 2023 revised IgG4-related cardiovascular/retroperitoneal disease criteria show significantly enhanced sensitivity while preserving high specificity, achieved through the inclusion of new items in radiologic, pathological, and extra-cardiovascular/retroperitoneal organ categories.

Corresponding author

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