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
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- Mizushima Ichiro
- Department of Nephrology and Rheumatology, Kanazawa University Hospital
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- Morikage Noriyasu
- Division of Vascular Surgery, Department of Surgery and Clinical Science, Yamaguchi University Graduate School of Medicine
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- Ito Eisaku
- Division of Vascular Surgery, Department of Surgery, The Jikei University School of Medicine
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- Kasashima Fuminori
- Department of Cardiovascular Surgery, Kanazawa Medical Center
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- Matsumoto Yasushi
- Department of Cardiovascular Surgery, Kanazawa Medical Center
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- Sawa Naoki
- Department of Nephrology and Rheumatology, Toranomon Hospital
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- Yoshifuji Hajime
- Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University
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- Saeki Takako
- Department of Internal Medicine, Nagaoka Red Cross Hospital
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- Shintani-Domoto Yukako
- Department of Diagnostic Pathology, Nippon Medical School Hospital
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- Shimada Shogo
- Department of Cardiac Surgery, The University of Tokyo
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- Takayama Toshio
- Division of Vascular Surgery, Department of Surgery, Graduate School of Medicine, The University of Tokyo
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- Amiya Eisuke
- Department of Cardiovascular Medicine, Graduate School of Medicine, The University of Tokyo
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- Ozawa Makiko
- Department of Internal Medicine, Matsumoto Dental University Hospital
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- Takahashi Masaaki
- Department of Radiology, Shinshu University School of Medicine
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- Fujinaga Yasunari
- Department of Radiology, Shinshu University School of Medicine
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- Katsumata Takahiro
- Department of Thoracic and Cardiovascular Surgery, Osaka Medical and Pharmaceutical University
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- Obitsu Yukio
- Department of Vascular Surgery, International University of Health and Welfare Mita Hospital
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- Izawa Atsushi
- School of Health Sciences, Shinshu University
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- Kanno Hiroyuki
- Department of Pathology, Shinshu University School of Medicine
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- Oyama-Manabe Noriko
- Department of Radiology, Jichi Medical University Saitama Medical Center
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- Ishizaka Nobukazu
- Health Check-up Center, Doai Memorial Hospital
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- Nagasawa Tasuku
- Division of Nephrology, Endocrinology, and Vascular Medicine, Tohoku University Hospital
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- Takahashi Hiroki
- Department of Rheumatology and Clinical Immunology, Sapporo Medical University School of Medicine
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- Ohki Takao
- Division of Vascular Surgery, Department of Surgery, The Jikei University School of Medicine
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- Kawano Mitsuhiro
- Department of Nephrology and Rheumatology, Kanazawa University Hospital
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- Kasashima Satomi
- Department of Clinical Laboratory Science, Graduate School of Health Science, Kanazawa University
説明
<p>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.</p><p>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.</p><p>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.</p>
収録刊行物
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- Circulation Journal
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Circulation Journal advpub (0), 2024-03-16
一般社団法人 日本循環器学会