Zhang Yudi, Shen Tian, Gao Sihao, Yan Junxia, Shi Jingcheng, Tang Xuemei, Wang Mo, Yang Jun, Yu Haiguo, Mao Huawei, Shuai Lanjun, Li Yongzhen, Cao Yan, Li Xiaoyan, Wang Ying, Liu Qian, Song Hongmei, Wu Xiaochuan
Department of Pediatrics, The Second Xiangya Hospital, Central South University, Changsha, Hunan, China.
Department of Pediatrics, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China.
Front Immunol. 2025 Jul 17;16:1611349. doi: 10.3389/fimmu.2025.1611349. eCollection 2025.
This study evaluated the diagnostic accuracy of the 2012 SLICC and 2019 EULAR/ACR criteria in Chinese cSLE patients and aimed to develop an optimized classification schema based on the 2019 EULAR/ACR criteria, specifically tailored for cSLE.
Data from cSLE and control cases were extracted from the CAPRID database. Gold-standard diagnosis were established by consensus among 43 rheumatologists (≥80% agreement). From 1,390 consensus cases, a random selection of 1,045 cases (512 cSLE/533 non-cSLE) were allocated to derivation (n=522) and validation (n=523) cohorts. The 2012 SLICC and 2019 EULAR/ACR criteria were evaluated in the total cohort. Multiple optimization schemes were then developed through LASSO regression with expert consultation in the derivation cohort. All potential optimization schemes underwent validation in the validation cohort, from which the optimal scheme was selected and further evaluated in an ANA-positive subgroup.
The 2012 SLICC criteria demonstrated sensitivity of 96.7% and specificity of 96.5%, while the 2019 EULAR/ACR criteria had sensitivity of 95.3% and specificity of 97.8%, with an optimal total score threshold of 10. When both the non-scarring alopecia and arthritis criteria were removed alongside the redefined urinary protein criterion, specificity significantly improved to 99.3% ( < 0.05), while sensitivity remained unaffected at 94.1% ( = 0.210). In the ANA-positive cohort, the optimized integrated scheme significantly improved specificity (97.7% vs. 86.4%, = 0.012) while maintaining comparable sensitivity (96.2% vs. 97.8%, = 0.138).
Both criteria performed well in Chinese cSLE patients. Optimizing the 2019 EULAR/ACR criteria by removing alopecia and arthritis criteria and modifying the urinary protein criterion enhanced specificity without compromising sensitivity.
本研究评估了2012年SLICC标准和2019年EULAR/ACR标准在中国儿童系统性红斑狼疮(cSLE)患者中的诊断准确性,并旨在基于2019年EULAR/ACR标准制定一种优化的分类方案,该方案专门针对cSLE进行了调整。
从CAPRID数据库中提取cSLE和对照病例的数据。由43名风湿病学家(≥80%的一致性)达成共识确定金标准诊断。从1390例达成共识的病例中,随机选择1045例(512例cSLE/533例非cSLE)分配到推导队列(n = 522)和验证队列(n = 523)。在整个队列中评估2012年SLICC标准和2019年EULAR/ACR标准。然后在推导队列中通过LASSO回归并咨询专家制定了多种优化方案。所有潜在的优化方案在验证队列中进行验证,从中选择最佳方案并在抗核抗体(ANA)阳性亚组中进一步评估。
2012年SLICC标准的敏感性为96.7%,特异性为96.5%,而2019年EULAR/ACR标准的敏感性为95.3%,特异性为97.8%,最佳总分阈值为10分。当非瘢痕性脱发和关节炎标准以及重新定义的尿蛋白标准被去除后,特异性显著提高至99.3%(P < 0.05),而敏感性保持在94.1%不受影响(P = 0.210)。在ANA阳性队列中,优化后的综合方案在保持相当敏感性(96.2%对97.8%,P = 0.138)的同时显著提高了特异性(97.7%对86.4%,P = 0.012)。
两种标准在中国cSLE患者中表现良好。通过去除脱发和关节炎标准以及修改尿蛋白标准来优化2019年EULAR/ACR标准可提高特异性而不影响敏感性。