Idiopathic pulmonary fibrosis (IPF) is a severe, progressive and irreversible lung disease constantly associated with a major vascular remodeling process. Endothelial colony-forming cells (ECFCs) are human vasculogenic cells proposed as a cell therapy product or liquid biopsy in vascular disorders. Since the link between IPF and thrombosis has been largely proposed, the aim of our study was to explore hypercoagulability states in ECFCs from patients with IPF. We performed Thrombin generation assay (TGA) in cord blood (CB)-ECFCs, peripheral blood (PB)-ECFCs and IPF-ECFCs. Endogenous thrombin potential and peak were higher in IPF-ECFCs compared to CB-ECFCs and PB-ECFCs. As thrombin generation in ECFCs was increased, we evaluated anticoagulant proteins expressed on ECFCs membrane and identified thrombomodulin and EPCR. We found a significant decrease of both anticoagulant proteins at membrane using flow cytometry. This study is the first to examine ECFC thrombin generation in IPF. This new finding strongly argues for a role of ECFC in IPF pathophysiology and thrombotic related disorders in IPF.

特发性肺纤维化 (IPF) 是一种严重的、进行性的和不可逆的肺部疾病，经常与主要的血管重塑过程相关。内皮集落形成细胞 (ECFC) 是人类血管生成细胞，被提议用作血管疾病中的细胞治疗产品或液体活检。由于 IPF 与血栓形成之间的联系已被广泛提出，我们研究的目的是探索 IPF 患者 ECFC 的高凝状态。我们在脐带血 (CB)-ECFC、外周血 (PB)-ECFC 和 IPF-ECFC 中进行了凝血酶生成测定 (TGA)。与 CB-ECFC 和 PB-ECFC 相比，IPF-ECFC 中的内源性凝血酶电位和峰值更高。随着 ECFCs 中凝血酶生成的增加，我们评估了 ECFCs 膜上表达的抗凝蛋白并鉴定了血栓调节蛋白和 EPCR。我们使用流式细胞仪发现膜上的两种抗凝蛋白显着减少。这项研究是第一个检查 IPF 中 ECFC 凝血酶生成的研究。这一新发现有力地证明了 ECFC 在 IPF 病理生理学和 IPF 血栓相关疾病中的作用。