Endocan and Circulating Progenitor Cells in Women with Systemic Sclerosis: Association with Inflammation and Pulmonary Hypertension.

Alberto Lo Gullo,Javier Rodríguez-Carrio,Giuseppe Mandraffino,Davide Sciortino,Michele Scuruchi,Concetta Zito,Carmela Morace,Clemente Giuffrida,Davide Sinicropi,Maria Postorino,Romina Gallizzi,Giovanni Squadrito,Saverio Loddo

doi:10.3390/biomedicines9050533

Abstract

Background: Systemic sclerosis (SSc) is characterized by early vasculopathy and fibrosis in the skin, lungs, and other tissues. Vascular manifestations of SSc include Raynaud’s phenomenon, digital ulcers, and pulmonary artery hypertension (PAH). PAH is the second most common cause of mortality in SSc. Circulating CD34+ cells associated with cardiovascular health status in several conditions, including chronic immune-inflammatory disease. CD34+ cell numbers have been found inconstantly reduced in SSc. Endocan, a proteoglycan expressed by endothelial cells, was recently suggested as a marker of vascular stress. We tested the relationships among CD34+ cells, endocan, inflammatory markers, vitamin D levels, and clinical parameters in SSc patients with PAH. METHODS: Standard echocardiography was performed. Vitamin D levels, CD34+ cells, inflammatory markers, endocan plasma levels were determined in 36 female SSc patients (24 diffuse/12 limited) and 36 matched controls (HC). RESULTS: We found no difference in CD34+ and vitamin D levels in SSc as compared to controls; ESR, CRP, fibrinogen, endocan, sPAP were higher in SSc with respect to controls. We found a correlation between endocan and: CD34+ cells (r: −0.540, p = 0.002), pulmonary arterial pressure (sPAP) (r: 0.565, p < 0.001), tricuspid annular plane excursion (TAPSE) (r: −0.311, p < 0.01), and E/A ratio (r: −0.487, p < 0.001), but not with ejection fraction (r: −0.057, p = 0.785) in SSc. CD34+ cells correlate with fibrinogen (r: −0.619, p < 0.001), sPAP (r: −0.404, p = 0.011), E/A (r: 0.470, p < 0.005 in SSc. CONCLUSION: CD34+ cell number was significantly correlated with endocan levels and with sPAP in SSc; endocan and CD34+ progenitor cells might be suggested as a potential marker of disease status.

Highlights

Systemic sclerosis (SSc) is an autoimmune disease characterized by skin and internal organ fibrosis, as well as vascular abnormalities [1,2], including endothelial dysfunction and pulmonary arterial hypertension (PAH) [2,3]
The clinical characteristics of SSc patients were summarized in Table 2; we recorded disease duration, the pattern of SSc, the presence of anticentromere antibodies (ACA), anti-topoisomerase I (Anti-Scl70), antinuclear autoantibodies (ANA); pulmonary and musculoskeletal involvement as well as previous renal crisis and esophageal reflux were evaluated
SSc; 3topatients not haveor entries relating to the period considered; walked a distance 3, or were smokers, or 3 had started therapy; had wereneeded assigned ography parameters; 8 were excluded because we did not find the blood sample stored in our biowalked a distance

Summary

Introduction

Systemic sclerosis (SSc) is an autoimmune disease characterized by skin and internal organ fibrosis, as well as vascular abnormalities [1,2], including endothelial dysfunction and pulmonary arterial hypertension (PAH) [2,3]. We found a correlation between endocan and: CD34+ cells (r: −0.540, p = 0.002), pulmonary arterial pressure (sPAP) (r: 0.565, p < 0.001), tricuspid annular plane excursion (TAPSE) (r: −0.311, p < 0.01), and E/A ratio (r: −0.487, p < 0.001), but not with ejection fraction (r: −0.057, p = 0.785) in SSc. CD34+ cells correlate with fibrinogen (r: −0.619, p < 0.001), sPAP (r: −0.404, p = 0.011), E/A

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Discussion

Conclusion