Rheumatology Division, Escola Paulista de Medicina, Universidade Federal de São Paulo, Rua Botucatu 740, 3º andar, São Paulo, SP, 04023-062, Brazil.
Rheumatol Int. 2017 Nov;37(11):1825-1833. doi: 10.1007/s00296-017-3787-1. Epub 2017 Aug 17.
The aim of this study was to validate the 2013 ACR/EULAR classification criteria for systemic sclerosis (SSc) in patients with SSc, including patients with early SSc. Fifty-six consecutive patients with early SSc (2001 LeRoy and Medsger criteria), 122 patients with established SSc (1980 ACR classification criteria), and 141 patients with SSc-like disorders were included in this cross-sectional study. The diagnostic performance of the 2013 ACR/EULAR criteria was compared with the 1980 ACR criteria in several subsets of patients. The performance of individual variables was also obtained. Receiver operating characteristic (ROC) curves and optimal cut-off values were computed. The sensitivity and specificity in the whole cohort of 178 SSc patients were 77.6 and 98.5%, respectively, using the 2013 ACR/EULAR criteria and 68.5 and 100%, respectively, using the 1980 ACR criteria. Twenty-eight percent of the patients with early SSc met the 2013 ACR/EULAR criteria. Among the patients with early SSc, 53% of those who had Raynaud's phenomenon, abnormal capillaroscopy and positive SSc-related antibodies met the 2013 ACR/EULAR criteria. The area under the ROC curve was 0.975 (95% confidence interval 0.962-0.987). The best cut-off value for the total score was ≥8 (sensitivity 82%; specificity 97.9%). The individual variables with the highest specificity values were proximal skin thickening, sclerodactyly (specificity 100%), telangiectasia and SSc-related antibodies (specificity 98.6%). Raynaud's phenomenon had the best sensitivity (99.4%) but had low specificity (4.2%). In conclusion, the 2013 ACR/EULAR classification criteria showed high accuracy and increased sensitivity in the classification of patients with early SSc.
这项研究的目的是验证 2013 年 ACR/EULAR 系统性硬化症（SSc）分类标准在 SSc 患者中的适用性，包括早期 SSc 患者。本横断面研究纳入了 56 例早期 SSc 患者（2001 年 LeRoy 和 Medsger 标准）、122 例确诊 SSc 患者（1980 年 ACR 分类标准）和 141 例 SSc 样疾病患者。比较了 2013 年 ACR/EULAR 标准与 1980 年 ACR 标准在几组患者中的诊断性能。还获得了各个变量的性能。计算了受试者工作特征（ROC）曲线和最佳截断值。在 178 例 SSc 患者的整个队列中，使用 2013 年 ACR/EULAR 标准的敏感性和特异性分别为 77.6%和 98.5%，而使用 1980 年 ACR 标准的敏感性和特异性分别为 68.5%和 100%。28%的早期 SSc 患者符合 2013 年 ACR/EULAR 标准。在早期 SSc 患者中，53%的出现雷诺现象、异常毛细血管镜检和阳性 SSc 相关抗体的患者符合 2013 年 ACR/EULAR 标准。ROC 曲线下面积为 0.975（95%置信区间 0.962-0.987）。总分最佳截断值为≥8（敏感性 82%；特异性 97.9%）。特异性最高的个体变量是近端皮肤增厚、硬皮病样指（特异性 100%）、毛细血管扩张和 SSc 相关抗体（特异性 98.6%）。雷诺现象的敏感性最好（99.4%），但特异性较低（4.2%）。总之，2013 年 ACR/EULAR 分类标准在早期 SSc 患者的分类中具有较高的准确性和敏感性。
