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Circulating endothelial progenitor cells in systemic sclerosis: association with disease severity
  1. J Avouac (javouac{at}
  1. Rheumatology A department, Cochin Hospital, France
    1. F Juin (juin{at}
    1. INSEM U602, Paul Brousse Hospital, France
      1. J Wipff (julienwipff{at}
      1. Rheumatology A department, Cochin Hospital, France
        1. PO Couraud (couraud{at}
        1. Institut Cochin, Université Paris Descartes, CNRS (UMR 8104), France
          1. G Chiocchia (chiocchia{at}
          1. INSERM U567, Cochin Hospital, France
            1. A Kahan (andre.kahan{at}
            1. Rheumatology A department, Cochin Hospital, France
              1. C Boileau (catherine.boileau{at}
              1. U.V.S.Q University, Biochemistry, hormonology and molecular genetics department, Ambroise Paré Hosp, France
                1. G Uzan (uzan{at}
                1. INSEM U602, Paul Brousse Hospital, France
                  1. Y Allanore (yannick.allanore{at}
                  1. Rheumatology A department, Cochin Hospital, France


                    Background: Heterogeneous data have been reported regarding the detection and the number of circulating endothelial progenitor cells (EPCs) in systemic sclerosis (SSc).

                    Objective: We investigated the number of circulating EPCs using recent recommendations and we quantified their late outgrowth in patients with SSc and healthy controls.

                    Patients and methods: EPCs, defined as Lin-/7AAD-/CD34+/CD133+/VEGFR-2+ cells, were quantified in 50 SSc patients (mean age: 55±16 years, disease duration: 9±9 years) and 26 controls (mean age: 53±19 years) by cell sorting/flow cytometry and by counting late outgrowth colony-forming units (CFU).

                    Results: SSc patients displayed higher circulating EPC counts than controls (median 86 [5-282] vs. 49 [5-275]) EPCs for 1 million Lin- mononuclear cells; p=0.01). Lower EPC counts were associated with the higher Medsger’s severity score (p=0.01) and with the presence of past and/or current digital ulcers (p=0.026). There was no difference for the number of late outgrowth EPC-CFUs between SSc patients and controls in cell culture evaluation. The formation of colonies was associated with higher levels of circulating EPCs (p=0.02) and the number of colonies correlated with levels of EPCs (R=0.73, p=0.0004), validating our combination of FACS surface markers.

                    Conclusions: We quantified circulating EPCs with an accurate combination of markers herein validated. Our data demonstrate increased circulating EPC levels in SSc, supporting their mobilisation from bone marrow. Furthermore, the subset of patients with digital vascular lesions and high severity score displayed low EPC counts, suggesting increased homing at this stage. The predictive value of this biomarker now warrants further evaluation.

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