BMPR2 and pulmonary arterial hypertension: Vascular remodeling in PAH is confined to the lung and patients with the disease do not exhibit alterations in systemic blood pressure or peripheral vascular pathology.4 This observation indicates that inherent differences in the cellular behavior in the vascular beds could potentially be one of the possible reasons why global BMPR2 changes only lead to pathogenic remodeling of the pulmonary vasculature while the systemic vessels remain unaltered.