Sulli A, Burroni A, Tuccio M, Pizzorni C, Craviotto C, Ghio M, Basso M, Seriolo B, Cutolo M
Divisione di Reumatologia, Dipartimento di Medicina Interna, Università degli Studi di Genova, Genoa.
Reumatismo. 2004 Jan-Mar;56(1):36-45. doi: 10.4081/reumatismo.2004.36.
The aim of the present study was to demonstrate, by nailfold videocapillaroscopy (NVC), the existence of diagnostic and follow-up parameters of microvascular damage in systemic sclerosis (SS) (grouped in the "early", "active" and "late" NVC patterns). The presence of the different subsets of skin involvement (limSS and difSS), as well as the role of some specific serum autoantibodies in the expression of the NVC parameters were investigated.
245 consecutive SS patients were recruited and clinical data assessed. Antinuclear (ANA), antitopoisomerase I (Scl70) and anticentromere (ACA) antibodies were investigated in all patients.
Giant capillaries and hemorrhages were confirmed to be the earliest NVC finding in SS (diagnostic parameters). The loss of capillaries, along with ramified capillaries and vascular architectural disorganization were validated as parameters of progression of SS microangiopathy. Really, both Raynaud's phenomenon (RP) and SS duration were detected longer in patients with the "late" than in those with the "active" or the "early" NVC pattern. Patients affected by limSS were found to have shorter disease duration, as well as showed more frequently the "early" or the "active" NVC patterns. Conversely, patients affected by the difSS showed longer disease duration and mostly the presence of the "active" or "late" NVC pattern. The Scl70 positivity was lower in the patients showing the "early" than in those with the "active" and the "late" NVC patterns, whereas no significant correlation was found between the Scl70 presence and both RP and SS duration. The ACA positivity was higher in patients showing the "early" NVC pattern, as well as in patients with longer disease duration.
This study confirms that the identification of distinct NVC patterns may be useful to evaluate the severity and the stage of the SS microvascular damage. The presence of the Scl70 antibodies seems related to a more rapid progression of the SS microangiopathy. On the contrary, the presence of the ACA seems to be related to a slower progression of the SS microvascular damage. The SS peripheral microangiopathy is similar as in patients with limSS, as in those affected by difSS.
本研究旨在通过甲襞视频毛细血管镜检查(NVC),证明系统性硬化症(SS)微血管损伤的诊断和随访参数的存在(分为“早期”、“活动期”和“晚期”NVC模式)。研究了不同皮肤受累亚组(局限性SS和弥漫性SS)的存在,以及一些特定血清自身抗体在NVC参数表达中的作用。
招募了245例连续的SS患者并评估临床数据。对所有患者检测抗核抗体(ANA)、抗拓扑异构酶I抗体(Scl70)和抗着丝点抗体(ACA)。
巨型毛细血管和出血被确认为SS中最早的NVC表现(诊断参数)。毛细血管丧失以及分支状毛细血管和血管结构紊乱被确认为SS微血管病变进展的参数。实际上,“晚期”NVC模式患者的雷诺现象(RP)和SS病程均比“活动期”或“早期”NVC模式患者更长。局限性SS患者的病程较短,且更常表现为“早期”或“活动期”NVC模式。相反,弥漫性SS患者的病程较长,且大多表现为“活动期”或“晚期”NVC模式。表现为“早期”NVC模式的患者中Scl70阳性率低于“活动期”和“晚期”NVC模式患者,而Scl70的存在与RP和SS病程均无显著相关性。表现为“早期”NVC模式的患者以及病程较长的患者中ACA阳性率较高。
本研究证实,识别不同的NVC模式可能有助于评估SS微血管损伤的严重程度和阶段。Scl70抗体的存在似乎与SS微血管病变的更快进展有关。相反,ACA的存在似乎与SS微血管损伤的较慢进展有关。SS周围微血管病变在局限性SS患者和弥漫性SS患者中相似。