In the GRIPHON trial, in patients with PAH (almost 30% in both groups had PAH associated with connective tissue disease), the addition of selexipag (an oral selective non-prostanoid prostacyclin receptor agonist) was associated with a decreased risk of the primary composite endpoint of death or a complication related to PAH when compared to placebo. Here, PTGIR is linked to pulmonary arterial hypertension.