Controversies and advances in connective tissue disease‐related pulmonary arterial hypertension

Abstract

Controversies and advances in connective tissue diseaserelated pulmonary arterial hypertension Pulmonary arterial hypertension (PAH), a subcategory of pulmonary hypertension (PH), is a chronic and progressive disorder characterized by abnormal vascular proliferation and remodeling, vasoconstriction and thrombosis of the pulmonary vasculature, leading to elevated pulmonary vascular resistance (PVR), ultimately resulting in right heart failure and death. 1 Of all the connective tissue diseases (CTD), PAH occurs most commonly in systemic sclerosis (SSc) accounting for approximately 74% of all CTD-PAH cases, followed by systemic lupus erythematosus (SLE) and mixed connective tissue disease (MCTD), both of which account for 8% of CTD-PAH. 2