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The external validation of the 2019 ACR/EULAR classification criteria for IgG4-related disease in a large cohort from China

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机构: [1]Department of Rheumatology, Peking Union Medical College Hospital, National Clinical Research Center for Dermatologic and Immunologic Diseases, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, State Key Laboratory of Complex Severe and Rare Diseases, Chinese Academy of Medical Science & Peking Union Medical College, Beijing, China [2]Department of Rheumatology, Beijing Tongren Hospital, Capital Medical University, Beijing, China [3]Department of Rheumatology and Immunology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China [4]Department of Internal Medicine, Peking Union Medical College Hospital, Peking Union Medical College & Chinese Academy of Medical Science, Beijing, China [5]M.D. Program, Peking Union Medical College, Beijing, China
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关键词: IgG4-realted disease Criteria diagnosis

摘要:
To externally validate the performance of the 2019 American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) classification criteria for IgG4-related disease (IgG4-RD) within a cohort from China and to compare the criteria with the 2020 revised comprehensive diagnostic (RCD) criteria for IgG4-RD.This study included 875 IgG4-RD and 302 non-IgG4-RD cases (213 mimickers and 89 patients with other diseases). Using expert clinical judgment as the gold standard for diagnosis of IgG4-RD, the performance (sensitivity, specificity, area under the curve (AUC) of the 2019 ACR/EULAR criteria for IgG4-RD was evaluated. We also compared it with the 2020 RCD criteria.The 2019 ACR/EULAR classification criteria had a sensitivity of 76.6% (95% CI: 73.8% to 79.4%) and a specificity of 98.0% (96.0%-99.4%), an AUC of 0.873 (0.857-0.889) in the overall cohort. Those false negative cases under the 2019 ACR/EULAR classification criteria had significantly lower levels of serum IgG4, and fewer had pathological information, with a higher frequency in the involvement of those uncommon organs compared with the true positive cases. The cases judged as negative by the 2019 ACR/EULAR classification criteria yet judged as "definite" by the 2020 RCD criteria had more involvement of uncommon organs.The 2019 ACR/EULAR classification criteria for IgG4-RD show outstanding specificity and good sensitivity in real-world clinical practice. The 2020 RCD criteria are helpful for the diagnosis of IgG4-RD in clinical scenarios where IgG4-RD presents as involving an isolated organ, especially the unusual sites.Copyright © 2023. Published by Elsevier Inc.

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出版当年[2022]版:
大类 | 2 区 医学
小类 | 2 区 风湿病学
最新[2023]版:
大类 | 2 区 医学
小类 | 2 区 风湿病学
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出版当年[2021]版:
Q2 RHEUMATOLOGY
最新[2023]版:
Q1 RHEUMATOLOGY

影响因子: 最新[2023版] 最新五年平均 出版当年[2021版] 出版当年五年平均 出版前一年[2020版] 出版后一年[2022版]

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第一作者机构: [1]Department of Rheumatology, Peking Union Medical College Hospital, National Clinical Research Center for Dermatologic and Immunologic Diseases, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, State Key Laboratory of Complex Severe and Rare Diseases, Chinese Academy of Medical Science & Peking Union Medical College, Beijing, China [2]Department of Rheumatology, Beijing Tongren Hospital, Capital Medical University, Beijing, China
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通讯机构: [1]Department of Rheumatology, Peking Union Medical College Hospital, National Clinical Research Center for Dermatologic and Immunologic Diseases, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, State Key Laboratory of Complex Severe and Rare Diseases, Chinese Academy of Medical Science & Peking Union Medical College, Beijing, China [*1]Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases, Beijing, China
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