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GRIPHON 10-YEAR OPEN-LABEL EXTENSION

GRIPHON Trial Overview

The safety and efficacy of UPTRAVI® (selexipag) were demonstrated in a multicenter, double-blind, placebo-controlled, parallel-group, event-driven study in patients with symptomatic PAH (>98% WHO FC II or III). The primary endpoint was the time to first disease progression event.1,2*

  • Treatment with UPTRAVI® resulted in a 40% risk reduction† (99% CI: 22% to 54%; P<0.0001; HR 0.60) in disease progression compared with placebo (27% [155/574] vs 41.6% [242/582], respectively)1,2
  • Adverse reactions occurring more frequently (≥5%) on UPTRAVI® compared with placebo are headache, diarrhea, jaw pain, nausea, myalgia, vomiting, pain in extremity, and flushing1
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*The primary endpoint was the time to first progression event: a) death, b) hospitalization for PAH, c) need for lung transplantation or balloon atrial septostomy for worsening of PAH, d) parenteral prostanoid or chronic oxygen therapy, or e) other disease progression (decrease in 6MWD plus worsening or need of other therapy). †Hazard ratio based on primary endpoint events up to the end of treatment.

GRIPHON 10-Year OLE Overview

Limitations

These data are from a long-term follow-up and an open-label extension study. All analyses are descriptive only. Baseline characteristics were not balanced. These uncontrolled observations do not allow comparison with a control group not given UPTRAVI® and cannot be used to determine the long-term effect of UPTRAVI® on mortality.

Study Objectives

Assessments included patient characteristics, Kaplan-Meier survival estimates (assessed in the overall population, by individual maintenance dose, and by background therapy and time from diagnosis), and safety and tolerability.3

10-YEAR OLE: Overall Survival

Time from UPTRAVI® initiation to death up to EOT + 30 days3

Kaplan-Meier survival estimates (95% confidence intervals) are shown.

10-Year OLE: Survival by Time From Diagnosis

In triple-combination therapy, time from UPTRAVI® initiation to death up to EOT + 30 days3

Kaplan-Meier survival estimates (95% confidence intervals) are shown. Does not include 112 patients in the overall population who did not have a PAH-specific background therapy at baseline.

10-Year OLE: Survival by Dose

A low personal dose of UPTRAVI® (200 mcg to 400 mcg BID) is equally effective as higher personal doses (up to 1600 mcg BID) in a 10-year open-label extension study3

Time from UPTRAVI® initiation to death up to EOT + 30 days3

Kaplan-Meier survival estimates (95% confidence intervals) are shown. Does not include 8 patients in the overall population who were on an individualized maintenance dose of UPTRAVI® <200 mcg BID and 1 patient whose individualized maintenance dose of UPTRAVI® (700/900 mcg BID) did not meet the criteria for “medium” dose.

UPTRAVI® demonstrated a consistent safety profile for over 10 years3

10 years of data: The longest follow-up in a clinical study of any PAH therapy3

Most frequent adverse events‡
ADVERSE EVENT3UPTRAVI® N=574, n (%)
Headache390 (68)
Diarrhea265 (46)
Nausea209 (36)
Pulmonary arterial hypertension worsening203 (35)
Pain in jaw156 (27)
Death§126 (22)

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‡Occurring in ≥25% of patients.

§Up to end of treatment + 30 days; most common (>1%) reasons for death were PAH worsening (6%), right ventricular failure (4%), sudden death (2%), and cardiac arrest (1%).

6MWD=6-minute walk distance; BID=twice daily; CI=confidence interval; EOT=end of treatment; ERA=endothelin receptor antagonist; FC=Functional Class; GRIPHON=Prostacyclin (PGI2) Receptor Agonist In Pulmonary Arterial HypertensiON; HR=hazard ratio; OLE=open-label extension; PAH=pulmonary arterial hypertension; PDE5i=phosphodiesterase type 5 inhibitor; WHO=World Health Organization.

References: 1. UPTRAVI® (selexipag) full Prescribing Information. Actelion Pharmaceuticals US, Inc. 2. Sitbon O, Channick R, Chin KM, et al; GRIPHON Investigators. Selexipag for the treatment of pulmonary arterial hypertension. N Engl J Med. 2015;373:2522-2533. 3. McLaughlin VV, Howard L, Elwing J, et al. 10-year data on oral selexipag: long-term survival, safety, and dosing insights in pulmonary arterial hypertension (PAH) from the GRIPHON study and its open-label extension (OLE). Oral presentation presented at: 2025 International Society for Heart & Lung Transplantation (ISHLT); April 27-30, 2025; Boston, MA. 4. Humbert M, Kovacs G, Hoeper MM, et al; ESC/ERS Scientific Document Group. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: the Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS). Eur Heart J. 2022;43(38):3618-3731. 5. Zeng C, Liu J, Zheng X, et al. Prostaglandin and prostaglandin receptors: present and future promising therapeutic targets for pulmonary arterial hypertension. Respir Res. 2023;24(1):263. doi:10.1186/s12931-023-02559-3