Summary

Patients with systemic sclerosis pulmonary arterial hypertension (SSc-PAH) have a worse prognosis than those with idiopathic PAH. Although the specific mechanisms are not known, it is thought to be related to persistent right ventricular dysfunction and pulmonary vascular remodeling despite guideline-directed management with a single agent for PAH. This article discusses a 36-week, open-label, multicenter observational study evaluating upfront therapy with 2 drugs, tadalafil and ambrisentan, that target distinct pathways in treatment-naïve patients with SSc-PAH [NCT01178073; Gashouta MA et al. Am J Respir Crit Care Med].