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CXCR4^pos circulating progenitor cells coexpressing monocytic and endothelial markers correlating with fibrotic clinical features are present in the peripheral blood of patients affected by systemic sclerosis

¹ Department of Biomedical Sciences and Advanced Therapies, Hematology Section and BMT Unit, University of Ferrara-S.Anna Hospital, Ferrara
² Department of Clinical and Experimental Medicine, Section of Rheumatology, University of Ferrara-S.Anna Hospital, Ferrara, Italy

Correspondence: Francesco Lanza, Section of Hematology, BMT Unit, University of Ferrara-S.Anna Hospital, Corso Giovecca n. 203, 44100 Ferrara, Italy. E-mail:lanza.f{at}ospfe.it

There is still controversy regarding the role of circulating endothelial and progenitor cells (CECs/CEPs) in the pathogenesis of systemic sclerosis (SSc). Using a sequential Boolean gating strategy based on a 4-color flow cytometric protocol, an increased number of CD31^pos/CD184^pos(CXCR4)/CD34^pos/CD45^pos and CD31^pos/CD117^pos (c-kit-R) /CD34^pos/ CD45^pos hematopoietic circulating progenitor cells (HCPCs) was detected in SSc patients compared with healthy subjects. In SSc, no circulating mature and progenitor endothelial cells were observed, while an enhanced generation of erythroid progenitor cells was found to be correlated with the presence of CD117+ HCPCs. The presence of freshly detected CXCR4posHCPC was correlated either to the in vitro cultured spindle-shaped endothelial like cells (SELC) with an endo/myelomonocytic profile or to SDF-1 and VEGF serum level. These data are related to more fibrotic clinical features of the disease, thus supporting a possible role of these cells in fibrosis.

Key words: circulating hematopoietic progenitor cells, endothelial-like cells, monocytes, CXCR4, systemic sclerosis.