The 2019 American College of Rheumatology/European League Against Rheumatism Classification Criteria for IgG4‐Related Disease. Issue 1 (2nd December 2019)

• Record Type: Journal Article
• Authors: Wallace, Zachary S.; Naden, Ray P.; Chari, Suresh; Choi, Hyon; Della‐Torre, Emanuel; Dicaire, Jean‐Francois; Hart, Phil A.; Inoue, Dai; Kawano, Mitsuhiro; Khosroshahi, Arezou; Kubota, Kensuke; Lanzillotta, Marco; Okazaki, Kazuichi; Perugino, Cory A.; Sharma, Amita; Saeki, Takako; Sekiguchi, Hiroshi; Schleinitz, Nicolas; Stone, James R.; Takahashi, Naoki; Umehara, Hisanori; Webster, George; Zen, Yoh; Stone, John H.
• Other Names: Akamizu Takashi investigator.; Akiyama Mitsuhiro investigator.; Barra Lillian investigator.; Bateman Adrian investigator.; Blockmans Daniel investigator.; Brito‐Zeron Pilar investigator.; Campochiaro Corrado investigator.; Carruthers Mollie investigator.; Chari Suresh investigator.; Chiba Tsutomu investigator.; Choi Hyon investigator.; Cornell Lynn investigator.; Culver Emma investigator.; Darabian Saman investigator.; Torre Emanuel Della investigator.; Deshpande Vikram investigator.; Dong Lingli investigator.; Ebbo Mikael investigator.; Fernández‐Codina Andreu investigator.; Ferry Judith A. investigator.; Fragkoulis George investigator.; Frost Fabian investigator.; Frulloni Luca investigator.; Hart Phil A. investigator.; Hernandez‐Molina Gabriela investigator.; Inoue Dai investigator.; Ji Haihan investigator.; Keat Karuna investigator.; Kamisawa Terumi investigator.; Kawa Shigeyuki investigator.; Kawano Mitsuhiro investigator.; Khosroshahi Arezou investigator.; Kobayashi H. investigator.; Kodama Yuzo investigator.; Kubo Satoshi investigator.; Kubota Kensuke investigator.; Lanzillotta Marco investigator.; Leng Haiyang investigator.; Lerch Markus investigator.; Liu Yanying investigator.; Liu Zhifu investigator.; Löhr Matthias investigator.; Martin‐Nares Eduardo investigator.; Martinez‐Valle Ferran investigator.; Marvisi Chiara investigator.; Masaki Yasufumi investigator.; Matsui Shoko investigator.; Mizushima Ichiro investigator.; Naden Ray P. investigator.; Nakamura Seiji investigator.; Nordeide Jan investigator.; Notohara Kenji investigator.; Okazaki Kazuichi investigator.; Paira Sergio investigator.; Perugino Cory A. investigator.; Popovic Jovan investigator.; Ramos‐Casals Manel investigator.; Rosenbaum James investigator.; Ryu Jay investigator.; Saeki Takako investigator.; Sato Yasuharu investigator.; Schleinitz Nicolas investigator.; Sekiguchi Hiroshi investigator.; Sharma Amita investigator.; Sokol Evgeniya V. investigator.; Stone James R. investigator.; Stone John H. investigator.; Sun Wenwu investigator.; Takahashi Hiroki investigator.; Takahashi Naoki investigator.; Takahira Masayuki investigator.; Tanaka Yoshiya investigator.; Umehara Hisanori investigator.; Vaglio Augusto investigator.; Villamil Alejandra investigator.; Wada Yoko investigator.; Wallace Zachary S. investigator.; Webster George investigator.; Yamada Kazunori investigator.; Yamamoto Motohisa investigator.; Yi Joanne investigator.; Yi Yinlan investigator.; Zamboni Giuseppe investigator.; Zen Yoh investigator.; Zhang Wen investigator.;
• Is Part Of: Arthritis & rheumatology. Volume 72:Issue 1(2020)
• Page Start: 7
• Page End: 19
• Publication Date: 2019-12-02
• Subjects: Arthritis -- Periodicals; Rheumatism -- Periodicals; 616.72
• DOI: 10.1002/art.41120
• Languages: English
• ISSNs: 2326-5191

Abstract

Abstract : Objective: IgG4‐related disease (IgG4‐RD) can cause fibroinflammatory lesions in nearly any organ. Correlation among clinical, serologic, radiologic, and pathologic data is required for diagnosis. This work was undertaken to develop and validate an international set of classification criteria for IgG4‐RD. Methods: An international multispecialty group of 86 physicians was assembled by the American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR). Investigators used consensus exercises, existing literature, derivation and validation cohorts of 1, 879 subjects (1, 086 cases, 793 mimickers), and multicriterion decision analysis to identify, weight, and test potential classification criteria. Two independent validation cohorts were included. Results: A 3‐step classification process was developed. First, it must be demonstrated that a potential IgG4‐RD case has involvement of at least 1 of 11 possible organs in a manner consistent with IgG4‐RD. Second, exclusion criteria consisting of a total of 32 clinical, serologic, radiologic, and pathologic items must be applied; the presence of any of these criteria eliminates the patient from IgG4‐RD classification. Third, 8 weighted inclusion criteria domains, addressing clinical findings, serologic results, radiology assessments, and pathology interpretations, are applied. In the first validation cohort, a threshold of 20 points had a specificity of 99.2% (95% confidence interval [95% CI] 97.2–99.8%) and a sensitivity of 85.5% (95% CI 81.9–88.5%). In the second, the specificity was 97.8% (95% CI 93.7–99.2%) and the sensitivity was 82.0% (95% CI 77.0–86.1%). The criteria were shown to have robust test characteristics over a wide range of thresholds. Conclusion: ACR/EULAR classification criteria for IgG4‐RD have been developed and validated in a large cohort of patients. These criteria demonstrate excellent test performance and should contribute substantially to future clinical, epidemiologic, and basic science investigations.