Key result
Long-term selexipag shows ~74% five-year survival in patients with PAH.
Why the study?
Longer-term survival, safety, and dosing patterns for oral selexipag in pulmonary arterial hypertension remained to be characterized across the complete observation period of GRIPHON and its open-label extension.
What are the long-term survival and safety outcomes of oral selexipag in patients with pulmonary arterial hypertension?
Cohort (n=953)
Open-label
Randomized in parent trial
What are the long-term survival and safety outcomes of oral selexipag in patients with pulmonary arterial hypertension?
Long-term treatment with oral selexipag in PAH patients demonstrates a 5-year survival rate of 74%, with a safety profile consistent with known prostacyclin effects.
May support long-term selexipag in PAH; extends observational data but leaves open need for randomized confirmation.
BACKGROUND: Selexipag, an oral prostacyclin receptor agonist, significantly reduced morbidity/mortality risk versus placebo for patients with pulmonary arterial hypertension (PAH) in GRIPHON. Five-year survival estimates in real-world clinical practice for PAH patients are 57%. With its open-label extension (OLE), GRIPHON presents the longest follow-up in a PAH trial to date. METHODS: Two main analysis sets were evaluated as follows: selexipag-treated patients i.e., those receiving selexipag in GRIPHON (NCT01106014) or OLE (NCT01112306) and selexipag-long term patients, i.e., those randomized to selexipag in GRIPHON, irrespective of entry into its OLE. Safety was analyzed in both. On-treatment survival was analyzed (Kaplan-Meier [KM] estimates; 95% confidence intervals [CI]) in the overall selexipag long-term patients and by subgroups (individualized maintenance dose, PAH-specific combination therapy and time from diagnosis, and 4-strata risk category), to end of treatment + 30 days. RESULTS: Overall, 953 selexipag-treated patients were identified; 574 were selexipag long-term patients. For the latter, median follow-up time from selexipag initiation was 54 months and median exposure duration was 35.8 months (total selexipag exposure: 2105.5 patient-years). KM (95% CI) survival estimates at 5 years were 74% (69-78) overall (n = 176) and 79% (67-87) when selexipag was initiated with an endothelin receptor antagonist and phosphodiesterase 5 inhibitor (n = 26). The most frequently reported adverse events (AEs) were related to known prostacyclin-related effects or underlying disease; AEs leading to selexipag discontinuation were reported in 223 (39%) patients. CONCLUSIONS: These comprehensive, long-term safety and survival data for selexipag provide the longest follow-up period to date for a PAH therapy. Selexipag's safety profile, up to 10 years in almost 1000 patients, was consistent with previous observations.
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McLaughlin et al. (2025) conducted a cohort in Pulmonary arterial hypertension (PAH) (n=953). Selexipag was evaluated on On-treatment survival at 5 years (95% CI 69-78). Long-term treatment with selexipag in patients with pulmonary arterial hypertension demonstrated a 5-year survival estimate of 74% (95% CI 69-78).
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