Rheumatology Unit, Department of Clinical and Molecular Sciences, Marche Polytechnic University, Ancona, Italy.
Scleroderma Clinic, Rheumatology Unit, Sapienza University of Rome, Rome, Italy.
Rheumatology (Oxford). 2024 May 3;63(6):1616-1623. doi: 10.1093/rheumatology/kead445.
To evaluate differences in nailfold videocapillaroscopy (NVC) findings between SSc patients with and without a diagnosis of pulmonary arterial hypertension (PAH).
One hundred and ten SSc patients were enrolled in this cross-sectional, case-control, multicentre study. Patients were divided into cases (SSc-PAH confirmed by right heart catheterization) and controls (SSc-nonPAH with low probability of PAH). NVC patterns (early, active and late) and morphological parameters (microvascular density, non-specific abnormalities, giant capillaries, micro-haemorrhages, avascular areas) were considered using a semiquantitative scoring system.
SSc-PAH patients showed higher frequencies of late pattern (P < 0.01), non-specific abnormalities (P < 0.01), lower capillary density (P < 0.01), higher avascular areas (P < 0.01) and a higher mean NVC score (P < 0.01). Contrarily, the early/active pattern (P < 0.01) and a higher rate of micro-haemorrhages (P = 0.04) were more frequent in non-PAH patients. By a multivariate analysis, SSc-PAH patients, compared with non-PAH, had more non-specific abnormalities [27/55, 49.1% vs 10/55, 18.2%; adjusted odd ratio (OR) 16.89; 95% CI: 3.06, 93.16], a lower capillary density (grade 3, 20/55, 36.4% vs 5/55, 9.1%; adjusted OR 38.33; 95% CI: 2.34, 367.80) and avascular areas (18/55, 32.7% vs 10/55, 18.2%; adjusted OR 16.90; 95% CI: 2.64, 44.35). A correlation was found between the mean pulmonary arterial pressure and avascular areas (P < 0.01), capillary density (P < 0.01) and non-specific abnormalities (P < 0.01). A clinical model including the NVC variables may be able to predict a diagnosis of PAH.
Our results indicate that the distinctive peripheral microcirculatory injury of SSc, i.e. capillary loss and morphological abnormalities, appear more severe and pronounced in patients with SSc-PAH.
评估有和无肺动脉高压（PAH）诊断的系统性硬化症（SSc）患者的甲襞毛细血管镜（NVC）检查结果差异。
本研究为一项横断面、病例对照、多中心研究，共纳入 110 例 SSc 患者。患者分为病例组（经右心导管检查确诊的 SSc-PAH）和对照组（SSc-非 PAH 且 PAH 可能性低）。使用半定量评分系统评估 NVC 模式（早期、活跃和晚期）和形态学参数（微血管密度、非特异性异常、巨毛细血管、微出血、无血管区）。
SSc-PAH 患者晚期模式（P<0.01）、非特异性异常（P<0.01）、毛细血管密度较低（P<0.01）、无血管区较高（P<0.01）和平均 NVC 评分较高（P<0.01）的发生率更高。相反，非 PAH 患者中早期/活跃模式（P<0.01）和较高的微出血发生率（P=0.04）更为常见。多变量分析显示，与非 PAH 患者相比，SSc-PAH 患者的非特异性异常更多[27/55，49.1%比 10/55，18.2%；调整后比值比（OR）16.89；95%置信区间：3.06，93.16]，毛细血管密度较低（3 级，20/55，36.4%比 5/55，9.1%；调整后 OR 38.33；95%置信区间：2.34，367.80）和无血管区较大（18/55，32.7%比 10/55，18.2%；调整后 OR 16.90；95%置信区间：2.64，44.35）。平均肺动脉压与无血管区（P<0.01）、毛细血管密度（P<0.01）和非特异性异常（P<0.01）呈正相关。包含 NVC 变量的临床模型可能能够预测 PAH 诊断。
我们的结果表明，SSc 患者外周微循环损伤更为严重，即毛细血管丧失和形态异常，在 SSc-PAH 患者中更为明显和严重。
