Objectives This study aimed to test the performance of the new ACR and EULAR criteria, that include ANA positivity as entry criterion, in JSLE. Methods Performance of the ACR/EULAR-2019 criteria were compared with Systemic Lupus International Collaborating Clinics (SLICC-2012), using data from children and young people (CYP) in the UK JSLE Cohort Study (n = 482), with the ACR-1997 criteria used as reference standard. An unselected cohort of CYP positive for ANA (n = 129) was used to calculate positive/negative predictive values of the criteria. Results At both first and last visits, the number of patients fulfilling the different classification criteria varied significantly (P < 0.001). The sensitivity of the SLICC-2012 criteria was higher when compared with that of the ACR/EULAR-2019 criteria at first and last visits (98% vs 94% for first visit, and 98% vs 96% for last visit; P < 0.001), when all available CYP were considered. The ACR/EULAR-2019 criteria were more specific when compared with the SLICC-2012 criteria (77% vs 67% for first visit, and 81% vs 71% for last visit; P < 0.001). Significant differences between the classification criteria were mainly caused by the variation in ANA positivity across ages. In the unselected cohort of ANA-positive CYP, the ACR/EULAR-2019 criteria produced the highest false-positive classification (6/129, 5%). Conclusion In CYP, the ACR/EULAR-2019 criteria are not superior to those of the SLICC-2012 or ACR-1997 criteria. If classification criteria are designed to include CYP and adult populations, paediatric rheumatologists should be included in the consensus and evaluation process, as seemingly minor changes can significantly affect outcomes.

中文翻译：

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JSLE 中 SLE 的 ACR/EULAR-2019 分类标准的敏感性和特异性有限？——来自英国 JSLE 队列研究的观察结果


目标 本研究旨在测试 JSLE 中新 ACR 和 EULAR 标准的性能，其中包括 ANA 阳性作为准入标准。方法 使用英国 JSLE 队列研究 (n = 482) 中儿童和青少年 (CYP) 的数据，将 ACR/EULAR-2019 标准的表现与系统性狼疮国际合作诊所 (SLICC-2012) 进行比较，其中 ACR- 1997 年标准用作参考标准。使用未选择的 ANA CYP 阳性队列 (n = 129) 来计算标准的阳性/阴性预测值。结果 在第一次和最后一次就诊时，满足不同分类标准的患者数量差异显着 (P < 0.001)。首次和末次就诊时，SLICC-2012 标准的敏感性高于 ACR/EULAR-2019 标准（首次就诊为 98% vs 94%，末次就诊为 98% vs 96%；P < 0.001），当考虑所有可用的 CYP 时。与 SLICC-2012 标准相比，ACR/EULAR-2019 标准更加具体（首次就诊为 77% vs 67%，上次就诊为 81% vs 71%；P < 0.001）。分类标准之间的显着差异主要是由于不同年龄阶段 ANA 阳性率的差异造成的。在未选择的 ANA 阳性 CYP 队列中，ACR/EULAR-2019 标准产生了最高的假阳性分类（6/129，5%）。结论 在CYP中，ACR/EULAR-2019标准并不优于SLICC-2012或ACR-1997标准。如果分类标准旨在包括 CYP 和成年人群，则应将儿科风湿病专家纳入共识和评估过程中，因为看似微小的变化可能会显着影响结果。