Complementary experimental and genetic models of PAH reveal therapeutic anti-inflammatory activities of the activin receptor type IIA-fragment crystallizable (ActRIIA-Fc) fusion protein that, together with its known anti-proliferative effects on vascular cell types, could underline the clinical activity of sotatercept as either monotherapy or an add-on to current PAH therapies [41]. Here, ACVR2A is linked to pulmonary arterial hypertension.