Idiopathic and connective tissue disease-associated pulmonary arterial hypertension share common pathogenic mechanisms, including endothelial dysfunction and an autoimmune signature, but differ in specific autoantibody profiles.
This review highlights the shared autoimmune signatures and distinct pathogenic features between idiopathic and connective tissue disease-associated pulmonary arterial hypertension, suggesting potential novel therapeutic targets.
Pre-capillary pulmonary arterial hypertension (PAH) is hemodynamically characterized by a mean pulmonary arterial pressure (mPAP) ≥ 20 mmHg, pulmonary capillary wedge pressure (PAWP) ≤15 mmHg and pulmonary vascular resistance (PVR) > 2. PAH is classified in six clinical subgroups, including idiopathic PAH (IPAH) and PAH associated to connective tissue diseases (CTD-PAH), that will be the main object of this review. The aim is to compare these two PAH subgroups in terms of epidemiology, histological and pathogenic findings in an attempt to define disease-specific features, including autoimmunity, that may explain the heterogeneity of response to therapy between IPAH and CTD-PAH.
Favoino et al. (2024) conducted a review in Pulmonary arterial hypertension (PAH). Idiopathic and connective tissue disease-associated pulmonary arterial hypertension share common pathogenic mechanisms, including endothelial dysfunction and an autoimmune signature, but differ in specific autoantibody profiles.