Liu Zheng, Nie Yuxue, Peng Yu, Lu Hui, Zhang Panpan, Li Jieqiong, Sun Ruijie, Li Jingna, Sun Boyuan, He Xin, Zhou Jiaxin, Fei Yunyun, Peng Linyi, Zhang Wen, Zeng Xiaofeng
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; Department of Rheumatology, Beijing Tongren Hospital, Capital Medical University, Beijing, China.
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.
Semin Arthritis Rheum. 2023 Aug;61:152202. doi: 10.1016/j.semarthrit.2023.152202. Epub 2023 Mar 29.
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.
在一个中国队列中对2019年美国风湿病学会（ACR）/欧洲抗风湿病联盟（EULAR）IgG4相关疾病（IgG4-RD）分类标准的性能进行外部验证，并将该标准与2020年修订的IgG4-RD综合诊断（RCD）标准进行比较。
本研究纳入了875例IgG4-RD病例和302例非IgG4-RD病例（213例模仿者和89例其他疾病患者）。以专家临床判断作为IgG4-RD诊断的金标准，评估2019年ACR/EULAR IgG4-RD标准的性能（敏感性、特异性、曲线下面积（AUC））。我们还将其与2020年RCD标准进行了比较。
2019年ACR/EULAR分类标准在整个队列中的敏感性为76.6%（95%CI：73.8%至79.4%），特异性为98.0%（96.0%-99.4%），AUC为0.873（0.857-0.889）。2019年ACR/EULAR分类标准下的那些假阴性病例血清IgG4水平显著较低，病理信息较少，与真阳性病例相比，罕见器官受累频率更高。2019年ACR/EULAR分类标准判断为阴性但2020年RCD标准判断为“确诊”的病例罕见器官受累更多。
2019年ACR/EULAR IgG4-RD分类标准在实际临床实践中显示出出色的特异性和良好的敏感性。2020年RCD标准有助于在IgG4-RD表现为孤立器官受累尤其是不常见部位受累的临床情况下诊断IgG4-RD。
