Fig. 2.

Schematic representation of molecular mechanisms participating in vascular remodeling that promote PH in PF patients. Depicted in yellow, angiogenesis and endothelial dysfunction are known to be dysregulated in PF patients and to participate in PH development, making PF a fertile ground for PH development. Depicted in green, Adenosine and BMPR2 are known to be dysregulated in PF patients and to be further impaired in patients with PF-PH. Depicted in purple, HIF-1a, Slug and pulmonary arterial smooth muscle cells (SMC) proliferation are three hallmarks of patients with PF-PH