Department of Pediatrics, Renji Hospital, School of Medicine, Shanghai Jiao Tong University, Shan Dong Middle Road, Shanghai, 200001, People's Republic of China.
Department of Rheumatology, Renji Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, China.
Clin Rheumatol. 2022 Oct;41(10):2995-3003. doi: 10.1007/s10067-022-06293-x. Epub 2022 Jul 19.
To evaluate the performance of the 2019 European League Against Rheumatism/American College of Rheumatology (EULAR/ACR-2019) classification criteria for systemic lupus erythematosus (SLE) compared with the ACR-1997 and Systemic Lupus International Collaborating Clinics (SLICC) 2012 criteria in childhood-onset SLE (cSLE) and adult-onset SLE (aSLE) patients.
We conducted a retrospective study of SLE patients (221 children and 221 adults) and controls (214 children and 214 adults) with defined rheumatic diseases to establish each ACR-1997, SLICC-2012, and EULAR/ACR-2019 criterion fulfilled. Demographic, clinical, and laboratory features were evaluated through chart review.
For cSLE, sensitivities of ACR-1997, SLICC-2012, and EULAR/ACR-2019 criteria were 63.3%, 94.6%, and 98.2%, with specificities 99.5%, 98.6%, and 93.5%, respectively. For aSLE, sensitivities of ACR-1997, SLICC-2012, and EULAR/ACR-2019 criteria were 72.9%, 96.8%, and 99.1%, with specificities 97.2%, 92.5%, and 90.2%, respectively. When including only ANA positive patients, receiver operating characteristics analysis demonstrated that the cutoff value for EULAR/ACR-2019 criteria in cSLE and aSLE patients was 13 (sensitivity, 92.2%; specificity, 93.1%) and 10 (sensitivity, 99.1%; specificity, 85.1%), respectively. Twelve cSLE patients and seven aSLE patients only met the EULAR/ACR-2019 criteria, among whom eleven and four cases had single organ involvement, respectively.
The EULAR/ACR-2019 criteria showed similar sensitivity to cSLE and aSLE patients and was more sensitive than ACR-1997 and SLICC-2012 criteria, allowing earlier recognition of patients with single or major organ involvement. The adoption of a EULAR/ACR total score ≥ 13 in this study, instead of the initially proposed ≥ 10 points, could further improve the diagnostic performance of the EULAR/ACR-2019 criteria in cSLE. Key Points • Sensitivity of the EULAR/ACR-2019 criteria was high in both cSLE and aSLE patients. • The EULAR/ACR-2019 criteria allowed earlier recognition of patients with single or major organ damage. • The adoption of a EULAR/ACR total score ≥13 could further improve the diagnostic performance of the EULAR/ACR-2019 criteria in cSLE.
评估 2019 年欧洲抗风湿病联盟/美国风湿病学会（EULAR/ACR-2019）系统性红斑狼疮（SLE）分类标准与 ACR-1997 和系统性红斑狼疮国际合作临床中心（SLICC）2012 标准在儿童发病的 SLE（cSLE）和成人发病的 SLE（aSLE）患者中的表现。
我们对患有明确风湿性疾病的 SLE 患者（221 例儿童和 221 例成人）和对照者（214 例儿童和 214 例成人）进行了回顾性研究，以建立每个 ACR-1997、SLICC-2012 和 EULAR/ACR-2019 标准的满足情况。通过病历回顾评估人口统计学、临床和实验室特征。
对于 cSLE，ACR-1997、SLICC-2012 和 EULAR/ACR-2019 标准的敏感性分别为 63.3%、94.6%和 98.2%，特异性分别为 99.5%、98.6%和 93.5%。对于 aSLE，ACR-1997、SLICC-2012 和 EULAR/ACR-2019 标准的敏感性分别为 72.9%、96.8%和 99.1%，特异性分别为 97.2%、92.5%和 90.2%。当仅包括 ANA 阳性患者时，受试者工作特征分析表明，cSLE 和 aSLE 患者 EULAR/ACR-2019 标准的截断值分别为 13（敏感性，92.2%；特异性，93.1%）和 10（敏感性，99.1%；特异性，85.1%）。12 例 cSLE 患者和 7 例 aSLE 患者仅符合 EULAR/ACR-2019 标准，其中 11 例和 4 例分别为单一器官受累。
EULAR/ACR-2019 标准对 cSLE 和 aSLE 患者的敏感性相似，并且比 ACR-1997 和 SLICC-2012 标准更敏感，可更早识别出有单一或主要器官受累的患者。在本研究中采用 EULAR/ACR 总分≥13 而不是最初提出的≥10 分，可以进一步提高 EULAR/ACR-2019 标准在 cSLE 中的诊断性能。关键点• EULAR/ACR-2019 标准在 cSLE 和 aSLE 患者中的敏感性均较高。• EULAR/ACR-2019 标准可更早识别出有单一或主要器官损伤的患者。• 采用 EULAR/ACR 总分≥13 可进一步提高 EULAR/ACR-2019 标准在 cSLE 中的诊断性能。
