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Selexipag Delays PAH Progression in Patients with Connective Tissue Disease

Results of a subgroup analysis from the GRIPHON trial are promising for a hard-to-treat population.

Pulmonary arterial hypertension (PAH) is a dangerous complication of connective tissue disease (CTD), eg, systemic sclerosis, systemic lupus erythematosus, and has been historically difficult to treat. Recent studies suggest that treatment regimens that combine PAH therapies may be more effective in this population, but the studies are few. The current study analyzed a subgroup of patients with PAH-CTD enrolled in the GRIPHON trial (selective IP prostacyclin receptor agonist selexipag) to assess the impact of selexipag on PAH progression across CTD types. The study and results are summarized in this short slide show.