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Circulating cellular biomarkers in pulmonary arterial hypertension (PAH): relationship to clinical heterogeneity and therapeutic response

16 December 2021

Combined measurement of endothelial microvesicles and circulating progenitor cells could be a potential biomarker of endothelial dysfunction in PAH.

Pulmonary Arterial Hypertension (PAH) is a rare disease characterized by a sustained increase in pulmonary vascular resistance, which can lead to right ventricular failure and death. This increase is associated with remodeling of the vessel wall caused by proliferation of resident and inflammatory cells. Alterations that occur through different pathways and result in endothelial dysfunction (ED), which is a key phenomenon in the pathogenesis of PAH.

An investigation of the Respiratory research group of IDIBGI analyzed circulating levels of endothelial microvesicles (EMV) and progenitor cells (PC) in PAH patients and controls as biomarkers of pulmonary endothelial integrity. In addition, differences between PAH subtypes, in response to treatment and also in patients with scleroderma (SSc) and HIV without PAH were evaluated. Moreover, the research measured circulating levels of total, activated and circulating PCs and calculated the VME/PC ratio.

The results of the research, recently published in an article in the journal Cells, led by Dr. Olga Tura as first author, reflect how PAH patients showed a higher number of VMEs and a lower percentage of PCs compared to controls. The VME/PC ratio increased in patients with PAH and in patients with SSc or HIV without PAH. After treatment, no significant differences were observed.

Thus, the observational study concludes that PAH patients have altered vascular homeostasis, reflected in changes in circulating levels of MVO and PC, which do not recover with PAH-targeted therapy. Therefore, the combined measure of circulating VME and PC could be a potential biomarker of ED in PAH.



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