Riccieri V, Germano V, Alessandri C, Vasile M, Ceccarelli F, Sciarra I, Di Franco M, Spadaro A, Valesini G
Division of Rheumatology, Department of Clinical Medicine and Therapy, University of Rome "Sapienza", Rome, Italy.
Clin Exp Rheumatol. 2008 Nov-Dec;26(6):992-7.
Anti-endothelial cell antibodies (AECA) have been described in systemic sclerosis (SSc) but their clinical relevance is unclear.
Aim of this study was to measure serum levels of AECA in 62 SSc patients, examining the main clinical and laboratory features, including nailfold capillaroscopy (NC) abnormalities and looking for any significant association.
Fourteen patients (23%) were AECA positive. An "early" NC pattern was observed in 21 patients (34%), an "active" pattern in 24 (39%) and a "late" pattern in 17 cases (27%). In those patients with AECA, a "late" NC pattern was significantly more frequent respect to the "early" and "late" patterns (p<0.05); besides AECA serum levels were significantly higher in the "late" group of patients respect to the other two (p<0.04 and p<0.02 respectively), also showing a significantly more severe modified skin score (mSS) (> or =15) (p<0.04), while those cases with more aggressive NC patterns ("active" and "late") had a more frequent finding of arterial hypertension (p<0.05) and cardiac involvement (p<0.05) respect to those with "early" NC pattern.
Thus, advanced NC findings were more frequently found in those patients with higher levels of AECA and their contemporary presence may consent to identify specific SSc subsets i.e., those with higher skin scores and cardiovascular involvement. These data suggest that AECA may have a role in the progression of the endothelial damage and their presence and titer should be considered as an adjunctive risk factor for a more severe disease. We also confirm the diagnostic and prognostic validity for NC in SSc, underlying the importance for an accurate capillaroscopic assessment. The contemporary assessment of these two diagnostic tools can be useful to better define different subset of SSc patients.
抗内皮细胞抗体（AECA）已在系统性硬化症（SSc）中被描述，但其临床相关性尚不清楚。
本研究的目的是测量62例SSc患者血清中AECA的水平，检查主要的临床和实验室特征，包括甲襞毛细血管镜检查（NC）异常，并寻找任何显著关联。
14例患者（23%）AECA呈阳性。21例患者（34%）观察到“早期”NC模式，24例（39%）为“活动期”模式，17例（27%）为“晚期”模式。在AECA阳性患者中，“晚期”NC模式相对于“早期”和“活动期”模式显著更常见（p<0.05）；此外，“晚期”患者组的AECA血清水平显著高于其他两组（分别为p<0.04和p<0.02），同时改良皮肤评分（mSS）也显著更高（≥15）（p<0.04），而NC模式更具侵袭性的患者（“活动期”和“晚期”）相对于“早期”NC模式的患者，动脉高血压（p<0.05）和心脏受累（p<0.05）的发生率更高。
因此，在AECA水平较高的患者中更常发现晚期NC表现，它们的同时存在可能有助于识别特定的SSc亚组，即皮肤评分较高且有心血管受累的亚组。这些数据表明，AECA可能在内皮损伤的进展中起作用，其存在和滴度应被视为疾病更严重的辅助危险因素。我们还证实了NC在SSc中的诊断和预后有效性，强调了准确的毛细血管镜评估的重要性。这两种诊断工具的同时评估有助于更好地定义不同的SSc患者亚组。
