%0 Journal Article
%T 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.
%A Mizushima I
%A Morikage N
%A Ito E
%A Kasashima F
%A Matsumoto Y
%A Sawa N
%A Yoshifuji H
%A Saeki T
%A Shintani-Domoto Y
%A Shimada S
%A Takayama T
%A Amiya E
%A Ozawa M
%A Takahashi M
%A Fujinaga Y
%A Katsumata T
%A Obitsu Y
%A Izawa A
%A Kanno H
%A Oyama-Manabe N
%A Ishizaka N
%A Nagasawa T
%A Takahashi H
%A Ohki T
%A Kawano M
%A Kasashima S
%A  
%J Circ J
%V 0
%N 0
%D 2024 Mar 16
%M 38494710
%F 3.35
%R 10.1253/circj.CJ-24-0026
%X <B>BACKGROUND: </B>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.<BR><B>CONCLUSIONS: </B>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.