Andracco Romina, Irace Rosaria, Zaccara Eleonora, Vettori Serena, Maglione Wanda, Riccardi Antonella, Pignataro Francesca, Ferrara Roberta, Sambataro Domenico, Sambataro Gianluca, Vitali Claudio, Valentini Gabriele, Del Papa Nicoletta
UOC Day Hospital of Rheumatology, Department of Rheumatology, ASST G. Pini-CTO, Milan, Italy.
Rheumatology Unit, Department of Internal Medicine, 2nd University of Naples, Naples, Italy.
Arthritis Res Ther. 2017 Jun 13;19(1):133. doi: 10.1186/s13075-017-1354-5.
Some abnormalities in nailfold videocapillaroscopy (NVC), such as the presence of micro-haemorrhages (MHEs), micro-thromboses (MTs), giant capillaries (GCs) and reduction in the number of capillaries (nCs), suggest a disease activity (DA) phase in systemic sclerosis (SSc). In a previous paper, we showed that the number of micro-haemorrhages and micro-thromboses (the so-called NEMO score) was the NVC feature more closely associated with DA. The present study was aimed at validating the NEMO score as a measure of DA in patients with SSc.
Two cohorts of 122 and 97 patients with SSc who were referred to two different rheumatology units, one in Milan and one in Naples, respectively, constituted the validation cohorts. The NEMO score, the total number of GCs and the mean nCs per digit were the parameters defined in each patient by eight-finger NVC. An expert operator analysed the NVCs in each of the participating units. The European Scleroderma Study Group (ESSG) index was used to define the DA level in each patient at the time of NVC examination.
The NEMO score was the NVC parameter more strictly correlated with the ESSG score in both the Milan and Naples cohorts (p < 0.0001), and it was the only one among the NVC variables that gave a significant contribution in a logistic model where the ESSG score represented the dependent variable. ROC curve analysis confirmed that the NEMO score had the best performance in measuring DA. The AUC of the NEMO score was significantly greater than the AUCs obtained by plotting the sensitivity and specificity of the number of GCs and the mean nCs (p < 0.0001 in all cases). The NEMO score values that showed the best sensitivity-specificity balance in capturing patients with a relevant DA level were slightly higher in the Naples cohort than in the Milan cohort.
This study confirms that the presence of a certain number of MHEs and MTs in NVC may be considered a strong warning signal of a current phase of DA in patients with SSc.
甲襞视频毛细血管镜检查（NVC）中的一些异常，如微出血（MHEs）、微血栓形成（MTs）、巨型毛细血管（GCs）的存在以及毛细血管数量（nCs）减少，提示系统性硬化症（SSc）处于疾病活动（DA）期。在之前的一篇论文中，我们表明微出血和微血栓形成的数量（即所谓的NEMO评分）是与疾病活动最密切相关的NVC特征。本研究旨在验证NEMO评分作为SSc患者疾病活动度的一种测量方法。
分别来自米兰和那不勒斯两个不同风湿病科的122例和97例SSc患者组成了验证队列。NEMO评分、GCs总数和每根手指的平均nCs是通过八指NVC在每位患者中定义的参数。由一名专业操作人员对每个参与单位的NVC进行分析。欧洲硬皮病研究组（ESSG）指数用于定义NVC检查时每位患者的疾病活动水平。
在米兰和那不勒斯队列中，NEMO评分都是与ESSG评分相关性最紧密的NVC参数（p < 0.0001），并且在以ESSG评分为因变量的逻辑模型中，它是NVC变量中唯一做出显著贡献的变量。ROC曲线分析证实，NEMO评分在测量疾病活动度方面表现最佳。NEMO评分的曲线下面积（AUC）显著大于通过绘制GCs数量和平均nCs的敏感性和特异性所获得的AUC（在所有情况下p < 0.0001）。在捕捉具有显著疾病活动水平的患者时，显示出最佳敏感性-特异性平衡的NEMO评分值在那不勒斯队列中略高于米兰队列。
本研究证实，NVC中存在一定数量的MHEs和MTs可被视为SSc患者当前疾病活动期的一个强烈警示信号。
