Roles of bone marrow-derived cells in pathogenesis of pulmonary arterial hypertension associated with connective tissue diseases

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Pulmonary arterial hypertension(PAH) associated with systemic sclerosis(SSc) is one of intractable conditions with poor prognosis, but its pathogenic process is largely unknown. We have examined potential roles of endothelial progenitor cells(EPCs), which are involved in vascular repair, in the pathogenesis of PAH associated with SSc. As a result, EPCs in patients with SSc exhibited impaired vascular repair machinery, but had enhanced pro-fibrotic capacity. These EPC properties are the most prominent in SSc patients with PAH, and invasion capacity is augmented in EPCs derived from such patients. In summary, aberrant circulating EPC is a novel therapeutic target in PAH associated with SSc.