Pulmonary arterial hypertension, also known as group 1 pulmonary hypertension according to the World Health Organization categorization system, is a serious consequence of systemic sclerosis caused by pulmonary vascular involvement. The high mortality seen in systemic sclerosisassociated pulmonary arterial hypertension is likely due to impairment of right ventricular systolic function and the presence of other non-group-1 pulmonary hypertension phenotypes that may impede clinical response to pulmonary arterial hypertension-targeted therapy. This article outlines two areas of recent progress in the therapy of individuals with systemic sclerosis and pulmonary hypertension: the tolerability of pulmonary arterial hypertension-targeted therapy in patients with mild to severe interstitial lung disease, as well as the therapeutic importance of the antifibrotic impact of soluble guanylate cyclase stimulators seen in preclinical studies.