Ong Y Y, Nikoloutsopoulos T, Bond C P, Smith M D, Ahern M J, Roberts-Thomson P J
Department of Immunology, Allergy and Arthritis, Flinders Medical Centre, Adelaide, South Australia.
Asian Pac J Allergy Immunol. 1998 Jun-Sep;16(2-3):81-6.
Approximately 20% of patients with the limited form of scleroderma will develop pulmonary hypertension which is generally a late stage fatal complication. Why pulmonary hypertension occurs in this subset of patients is unknown and it has not been possible to predict which patients are at risk. Nailfold capillary dilatation, distortion and drop occurs universally in patients with scleroderma and is generally an early finding. The present study was conducted to investigate whether quantitative nailfold capillaroscopy could distinguish those limited scleroderma patients who have established pulmonary hypertension. Quantitative nailfold capillaroscopy was performed by Visual Image Analysis in 10 healthy subjects and 20 patients with limited scleroderma (18 centromere +ve), of whom 8 had established pulmonary hypertension. It was found that scleroderma patients with pulmonary hypertension had a significant reduction in capillary density compared with patients lacking this complication (p < 0.01). Patients with scleroderma have significantly more dilated capillaries than controls although no significant differences were observe between the two patient subgroups. The finding of reduced nailfold capillary density in scleroderma patients with established pulmonary hypertension has possible pathogenic significance and may allow detection of this subgroup at an early stage in their disease progression.
大约20%的局限性硬皮病患者会发展为肺动脉高压，这通常是一种晚期致命并发症。为什么这部分患者会发生肺动脉高压尚不清楚，也无法预测哪些患者有风险。甲襞毛细血管扩张、扭曲和缺失在硬皮病患者中普遍存在，且通常是早期表现。本研究旨在调查定量甲襞毛细血管镜检查能否区分已发生肺动脉高压的局限性硬皮病患者。对10名健康受试者和20名局限性硬皮病患者（18名着丝点抗体阳性）进行了定量甲襞毛细血管镜检查，其中8名已发生肺动脉高压。结果发现，与无此并发症的患者相比，发生肺动脉高压的硬皮病患者毛细血管密度显著降低（p<0.01）。硬皮病患者的扩张毛细血管明显多于对照组，尽管两个患者亚组之间未观察到显著差异。已发生肺动脉高压的硬皮病患者甲襞毛细血管密度降低这一发现可能具有致病意义，并可能有助于在疾病进展的早期阶段检测出该亚组患者。
