Pharmacological management of connective tissue disease-associated pulmonary arterial hypertension.

Despite significant advances over the past few decades with new medications and more aggressive treatment algorithms, patients with CTD-associated PAH are less likely to meet treatment targets and continue to have inferior survival compared to those with idiopathic PAH. This review concludes with our opinions on the current state of CTD-associated PAH, highlighting factors that lead to disparate outcomes compared to other forms of PAH, and a discussion on further improvements that may increase quality of life and survival for patients with CTD-associated PAH.