Circulating Endothelial Cells as a Marker of Ongoing Vascular Disease in Systemic Sclerosis

Nicoletta Del Papa, Gualtiero Colombo, Nicola Fracchiolla, Lorenza Mazzeo Moronetti, Francesca Ingegnoli, Wanda Maglione, Denise P. Comina, Claudio Vitali, Flavio Fantini, Agostino Cortelezzi

Research output: Contribution to journalArticle


Objective. Circulating endothelial cells (CECs) have been described in different conditions involving vascular injury. Vascular abnormalities play a key role in the pathogenesis of systemic sclerosis (SSc). The aim of this study was to search for the presence of CECs in patients with SSc and to evaluate their clinical associations and possible pathogenic role. Methods. The study cohort included 46 patients with SSc and 40 healthy controls. Five-parameter, 3-color flow cytometry was performed with a FACScan. CECs were defined as CD45 negative, CD34 positive, and P1H12 positive, and activated CECs were defined as CD45 negative and P1H12 positive, CD62 positive, or CD106 positive. Progenitors were identified as CD34 positive and CD133 positive. Results. Total and activated CEC counts were significantly higher in SSc patients compared with healthy controls and were positively correlated with the disease activity score. With respect to visceral involvement, significant correlation was observed between the CEC number and the severity of pulmonary hypertension. High levels of endothelial progenitors were observed in patients with SSc, and the counts were higher in the early stages of disease. Conclusion. The presence of CECs in patients with SSc may represent direct evidence of endothelial disease and may be a promising new clinical marker for active SSc. Notably, the association between CECs and pulmonary hypertension and impaired carbon monoxide diffusing capacity was evident in patients with limited cutaneous SSc only, suggesting an important role for CECs in this disease subset with prominent vascular changes. Detection of circulating endothelial progenitors may represent a response to vascular ischemia in early SSc, as an attempt at revascularization.

Original languageEnglish
Pages (from-to)1296-1304
Number of pages9
JournalArthritis and Rheumatism
Issue number4
Publication statusPublished - Apr 2004

ASJC Scopus subject areas

  • Immunology
  • Rheumatology

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