Molecular changes implicate angiogenesis and arterial remodeling in systemic sclerosis-associated and idiopathic pulmonary hypertension

Pulmonary hypertension (PH) is a common complication of systemic sclerosis (SSc) and a leading cause of mortality among patients with this disease. PH can also occur as an idiopathic condition (idiopathic pulmonary arterial hypertension, iPAH). We sought to investigate the transcriptomic alterations in PH vascular populations to understand cellular mechanisms underlying pathobiology of systemic sclerosis associated and idiopathic pulmonary hypertension. Overall design: We performed single-cell RNA sequencing on explanted lung tissue of patients with SSc-PH (n = 16), idiopathic pulmonary arterial hypertension (iPAH, n=3) and healthy controls (n=15).