Abstract
BackgroundThe association between systemic sclerosis and pulmonary arterial hypertension (PAH) is well recognized. Vascular endothelial growth factor (VEGF) has been reported to play an important role in pulmonary hypertension. The aim of the present study was to examine the relationship between systolic pulmonary artery pressure, clinical and functional manifestations of the disease and serum VEGF levels in systemic sclerosis.MethodsSerum VEGF levels were measured in 40 patients with systemic sclerosis and 13 control subjects. All patients underwent clinical examination, pulmonary function tests and echocardiography.ResultsSerum VEGF levels were higher in systemic sclerosis patients with sPAP ≥ 35 mmHg than in those with sPAP < 35 mmHg (352 (266, 462 pg/ml)) vs (240 (201, 275 pg/ml)) (p < 0.01), while they did not differ between systemic sclerosis patients with sPAP < 35 mmHg and controls. Serum VEGF levels correlated to systolic pulmonary artery pressure, to diffusing capacity for carbon monoxide and to MRC dyspnea score. In multiple linear regression analysis, serum VEGF levels, MRC dyspnea score, and DLCO were independent predictors of systolic pulmonary artery pressure.ConclusionSerum VEGF levels are increased in systemic sclerosis patients with sPAP ≥ 35 mmHg. The correlation between VEGF levels and systolic pulmonary artery pressure may suggest a possible role of VEGF in the pathogenesis of PAH in systemic sclerosis.
Highlights
The association between systemic sclerosis and pulmonary arterial hypertension (PAH) is well recognized
Pulmonary Function Tests Pulmonary function tests (PFTs) included measurement of forced expiratory volume in one second (FEV1), forced vital capacity (FVC), FEV1/FVC ratio, total lung capacity (TLC), residual volume, diffusing capacity for carbon monoxide (DLCO) and diffusing capacity for carbon monoxide adjusted for alveolar volume (DLCO/VA)
In the present study we have shown that serum Vascular endothelial growth factor (VEGF) levels were elevated in patients with Systemic sclerosis (SSc) and Systolic pulmonary artery pressure (sPAP) ≥ 35 mmHg compared to patients with SSc and sPAP < 35 mmHg and healthy controls
Summary
The association between systemic sclerosis and pulmonary arterial hypertension (PAH) is well recognized. Vascular endothelial growth factor (VEGF) has been reported to play an important role in pulmonary hypertension. The aim of the present study was to examine the relationship between systolic pulmonary artery pressure, clinical and functional manifestations of the disease and serum VEGF levels in systemic sclerosis. Systemic sclerosis (SSc) is a chronic connective tissue disease characterised by excessive collagen deposition in the skin and the internal organs and by immunologic abnormalities and vascular injury [1]. Significant pulmonary arterial hypertension (PAH) affects 15–20% of scleroderma patients and results in dyspnea, impaired exercise tolerance, and a high risk of death [4,5]. It has been reported that VEGF plays an important role in the development of pulmonary hypertension[13,14] and is strongly expressed in the normal pulmonary circulation and within the plexiform lesions of primary pulmonary hypertension[15]
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