Abstract

Background:Systemic sclerosis (SSc) is characterized by early vascular involvement and by varying degrees of fibrosis in skin, lungs, and other tissues. Vascular manifestations include Raynaud’s phenomenon, digital ulcers, and pulmonary hypertension (PAH). The prevalence of PAH is 7.85–13% in SSc and it is the second most common cause of mortality in SSc. Circulating CD34+ cell number is associated to cardiovascular health status in several chronic conditions, including inflammatory disease. CD34+ cell number was found inconstantly reduced in SSc. Endocan is a proteoglycan expressed by endothelial cells likely interacting with white blood cells, recently suggested as a marker of vascular stress.Objectives:to evaluate CD34+ cell number and endocan as a markers od cardiovascular involvement in SSc.Methods:Standard transthoracic echocardiography, Rodnan skin score were performed. Vitamin D levels, CD34+ cell number, markers of inflammation, red blood cells distribution width (RDW) and Endocan plasma levels were also determined.Results:37 female SSc patients (11 diffuse/26 limited) and 35 matched healthy controls (HC) were enrolled. CD34+ cell count was lower as compared to controls (2.3±0.73 vs 2.9±0.6; p<0.001), CRP and ESR were significantly higher in SSc patients than controls. Vitamin D3 was lower in SSc patients with respect to controls (18±4.5 vs 27.26±10.98; p<0.001), Rodnan skin score was 28.89±10.24. Endocan was two times greater than controls and serum endocan levels were higher in patients with diffuse Ssc as compared with limited ones. We found no correlation between Endocan and: Rodnan skin score, ESR, fibrinogen; we found a trend of correlation between Endocan and Vitamin D levels (r= -0.315), RDW (r= 0.310), CRP (r= 0.310), but statistical significance was not reached likely due to the small sample size. We found an association of PAPs with Endocan levels (rho= 0.442, p<0.01) and CD34+ count (-0.349, p<0.05. A significant correlation was also found between Endocan and CD34+ cell number (rho= -0.605, p<0.01).Conclusion:In our study population, we found a significant correlation between CD34+ cell number and Endocan plasma levels and PAPs; Endocan and CD34+ progenitor cells might be suggested as potential marker of pulmonary arterial hypertension in SSc patients.

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