Scheja A, Eskilsson J, Akesson A, Wollheim F A
Department of Rheumatology, University Hospital, Lund, Sweden.
J Rheumatol. 1994 Apr;21(4):639-42.
Microvascular abnormalities involving endothelial cell dysfunction occur as an early event in systemic sclerosis. We studied plasma concentrations of von Willebrand factor (vWf), a substance released from injured endothelial cells, and its relation to pulmonary and renal dysfunction.
vWf was determined by immunoelectrophoresis. Renal function was assessed by CrEDTA clearance, an accurate measure of glomerular filtration rate and pulmonary function by spirometry and carbon monoxide diffusing capacity. Pulmonary pressure was measured by Doppler cardiography.
In 22 patients with scleroderma, vWf concentrations related inversely to CrEDTA clearance. In contrast, no relationship between plasma vWf and pulmonary function was found; 9/10 patients with pulmonary hypertension and 9/12 patients with normal pulmonary pressure showed elevated levels of vWf. However in 5 patients with pulmonary hypertension without radiographic evidence of pulmonary fibrosis there was a strong correlation between plasma vWf and pulmonary pressure (rho = 0.90) which fell short of statistical significance due most likely to small population size.
Renal but not pulmonary dysfunction was associated with elevated plasma levels of vWf.
微血管异常涉及内皮细胞功能障碍，是系统性硬化症的早期事件。我们研究了血管性血友病因子（vWf）的血浆浓度，vWf是一种从受损内皮细胞释放的物质，以及它与肺和肾功能障碍的关系。
通过免疫电泳测定vWf。通过CrEDTA清除率评估肾功能，CrEDTA清除率是肾小球滤过率的准确指标，通过肺活量测定法和一氧化碳弥散能力评估肺功能。通过多普勒心动图测量肺动脉压力。
在22例硬皮病患者中，vWf浓度与CrEDTA清除率呈负相关。相比之下，未发现血浆vWf与肺功能之间存在关联；10例肺动脉高压患者中有9例以及12例肺动脉压力正常的患者中有9例vWf水平升高。然而，在5例无肺部纤维化影像学证据的肺动脉高压患者中，血浆vWf与肺动脉压力之间存在强相关性（rho = 0.90），但由于样本量小，最有可能未达到统计学意义。
肾功能障碍而非肺功能障碍与血浆vWf水平升高有关。
