Selexipag

Selexipag is a prostacyclin receptor (IP receptor) agonist that is structurally distinct from prostacyclin. Selexipag is hydrolyzed by carboxylesterase 1 to yield its active metabolite, which is approximately 37-fold as potent as selexipag.

• UPTRAVI is a prostacyclin receptor agonist indicated for the treatment of pulmonary arterial hypertension (PAH, WHO Group I): In adults to delay disease progression and reduce the risk of hospitalization for PAH. ( 1.1 ) In pediatric patients aged 2 years and older.ICD-10: I27.0

• Hypersensitivity to the active substance or to any of the excipients. Concomitant use of strong inhibitors of CYP2C8 (e.g., gemfibrozil) [see Drug Interactions (7.1) and Clinical Pharmacology (12.3) ] .contraindicated

Adult patients: UPTRAVI tablets starting dose: 200 mcg orally twice daily. Increase the dose by 200 mcg orally twice daily at weekly intervals to the highest tolerated dose up to 1,600 mcg orally twice daily. ( 2.1 ) Pediatric patients: See Full Prescribing Information for recommended starting dose, titration increments, and maximum allowed dose based on body weight category. ( 2.1 ) Maintenance dose is determined by tolerability. ( 2.1 ) Moderate hepatic impairment: Starting dose once daily, increase in increments of the starting dose once daily at weekly intervals to the maximum allowed or highest tolerated dose. ( 2.6 ) Adult patients: UPTRAVI for injection dose is determined by the patient's current dose of UPTRAVI tablets. Administer UPTRAVI for injection by intravenous infusion, twice daily. ( 2.2 ) See Full Prescribing Information for instructions on preparation and administration. ( 2.3 , 2.4 ) 2.1 Recommended Dosage and Administration for UPTRAVI Film-coated Tablets Adult Patients The recommended starting dosage of UPTRAVI tablets is 200 mcg given orally twice daily. Tolerability may be improved when taken with food [see Clinical Pharmacology (12.3) ] . Increase the dose in increments of 200 mcg orally twice daily, usually at weekly intervals, to the highest tolerated dose up to 1,600 mcg orally twice daily. If a patient reaches a dose that cannot be tolerated, the dose should be reduced to the previous tolerated dose. Swallow the UPTRAVI tablets whole. Do not split or crush the tablets.

Pulmonary edema in patients with pulmonary veno-occlusive disease. If confirmed, discontinue treatment. ( 5.1 ) 5.1 Pulmonary Edema with Pulmonary Veno-Occlusive Disease Should signs of pulmonary edema occur, consider the possibility of associated pulmonary veno-occlusive disease. If confirmed, discontinue UPTRAVI.

Adverse reactions in adult and pediatric patients occurring more frequently (≥5%) on UPTRAVI compared to placebo are headache, diarrhea, jaw pain, nausea, myalgia, vomiting, pain in extremity, and flushing. Additional adverse reaction occurring in pediatric patients more frequently (≥5%) on UPTRAVI compared to placebo is abdominal pain. ( 6.1 ) To report SUSPECTED ADVERSE REACTIONS, contact Actelion at 1-800-526-7736 or FDA at 1-800-FDA-1088 or www.fda.gov/medwatch. 6.1 Clinical Trial Experience Because clinical trials are conducted under widely varying conditions, adverse reaction rates observed in the clinical trials of a drug cannot be directly compared to rates in the clinical trials of another drug and may not reflect the rates observed in practice. UPTRAVI Tablets Adult Patients The safety of UPTRAVI tablets has been evaluated in a long-term, placebo-controlled study enrolling 1,156 adult patients with symptomatic PAH (GRIPHON study) [see Clinical Studies (14.1) ] . The exposure to UPTRAVI in this trial was up to 4.2 years with median duration of exposure of 1.4 years. Table 3 presents adverse reactions more frequent on UPTRAVI tablets than on placebo by ≥3%. Table 3: Adverse Reactions UPTRAVI Placebo Adverse Reaction N=575 N=577 Headache 65% 32% Diarrhea 42% 18% Jaw pain 26% 6% Nausea 33% 18% Myalgia 16% 6% Vomiting 18% 9% Pain in extremity 17% 8% Flushing 12% 5% Arthralgia 11% 8% Anemia 8% 5% Decreased appetite 6% 3% Rash 11% 8% These adverse reactions are more frequent during the dose titration phase.

Nursing mothers: Discontinue UPTRAVI or breastfeeding. ( 8.2 ) Severe hepatic impairment: Avoid use. ( 8.6 ) 8.1 Pregnancy Risk Summary There are no adequate and well-controlled studies with UPTRAVI in pregnant women. Animal reproduction studies performed with selexipag showed no clinically relevant effects on embryofetal development and survival. A slight reduction in maternal as well as in fetal body weight was observed when pregnant rats were administered selexipag during organogenesis at a dose producing an exposure to the active metabolite approximately 47 times that in humans at the maximum recommended human dose. No adverse developmental outcomes were observed with oral administration of selexipag to pregnant rabbits during organogenesis at exposures to the active metabolite up to 50 times the human exposure at the maximum recommended human dose. The estimated background risk of major birth defects and miscarriage for the indicated population is unknown. In the U.S. general population, the estimated background risk of major birth defects and miscarriage in clinically recognized pregnancies is 2–4% and 15–20%, respectively.

Metabolism

The pharmacokinetics of selexipag and its active metabolite have been studied primarily in healthy subjects. The pharmacokinetics of selexipag and the active metabolite, after both single- and multiple-dose oral administration, were dose-proportional up to a single dose of 800 mcg and multiple doses of up to 1,800 mcg twice daily.

Isolated cases of overdose in adults with UPTRAVI tablets up to 3,200 mcg were reported. Mild, transient nausea was the only reported consequence. In the event of overdose, supportive measures must be taken as required. Dialysis is unlikely to be effective because selexipag and its active metabolite are highly protein-bound.

• Dec 21, 2015NDANDA207947Actelion
• Jul 29, 2021NDANDA214275Actelion
• Dec 21, 2022ANDAANDA214302Zydus Lifesciences

1. 1Headache6,65926%
2. 2Diarrhoea5,11520%
3. 3Dyspnoea4,71019%
4. 4Nausea4,05916%
5. 5Fatigue2,56310%
6. 6Death2,5089.9%
7. 7Pain In Jaw2,4069.5%
8. 8Dizziness2,2739.0%
9. 9Pain2,1898.7%
10. 10Pain In Extremity2,1008.3%
11. 11Myalgia1,9677.8%
12. 12Vomiting1,9297.6%
13. 13Pneumonia1,7947.1%
14. 14Malaise1,7857.1%
15. 15Hypotension1,7036.7%

The 10 most recently updated of 37 ClinicalTrials.gov registrations naming Selexipag as an intervention. Registration is not evidence of efficacy or safety — reference crosswalk only.

• A Clinical Study of to Confirm the Doses of Selexipag in Children With Pulmonary Arterial Hypertension
  Active not recruiting · Phase 2 · Interventional · 63 enrolled · Actelion
  NCT03492177updated 2026-06-05
• A Study Providing Treatment Access in Participants With Pulmonary Hypertension Completing a Parent Study and Having no Other Option
  Recruiting · Phase 3 · Interventional · 280 enrolled · Actelion
  NCT05179876updated 2026-06-05
• A Study of Selexipag as Add-On Treatment to Standard of Care in Children With Pulmonary Arterial Hypertension
  Active not recruiting · Phase 3 · Interventional · 138 enrolled · Actelion
  NCT04175600updated 2026-06-05
• Pulmonary Hypertension: Intensification and Personalisation of Combination Rx
  Recruiting · Phase 4 · Interventional · 40 enrolled · Sheffield Teaching Hospitals NHS Foundation Trust
  NCT05825417updated 2026-06-05
• Effects of Selexipag in Adults With Raynaud's Phenomenon Secondary to Systemic Sclerosis
  Completed · Phase 2 · Interventional · 74 enrolled · Actelion
  NCT02260557updated 2026-06-03
• Individual Patient Expanded Access IND for Selexipag (Uptravi) in Participants With Non-healing Wound, Buerger's Disease
  Approved for marketing · Expanded access · Actelion
  NCT04914247updated 2026-03-27
• Long-Term Outcomes of Selexipag in Schistosomiasis-Associated Pulmonary Arterial Hypertension
  Not yet recruiting · Observational · 30 enrolled · Caio Júlio César dos Santos Fernandes
  NCT07453030updated 2026-03-05
• Patient-Reported Outcomes and Adherence After Transition From Inhaled Iloprost to Oral Selexipag in Pulmonary Arterial Hypertension
  Not yet recruiting · Observational · 32 enrolled · University of Sao Paulo General Hospital
  NCT07356375updated 2026-02-13
• Selexipag (ACT-293987) in Pulmonary Arterial Hypertension
  Completed · Phase 3 · Interventional · 1,156 enrolled · Actelion
  NCT01106014updated 2025-10-27
• Study of ACT-293987 (NS-304) in Pulmonary Arterial Hypertension (PAH)
  Completed · Phase 2 · Interventional · 43 enrolled · Actelion
  NCT00993408updated 2025-09-12

Frequently asked questions

How does Selexipag work?

Selexipag is a prostacyclin receptor (IP receptor) agonist that is structurally distinct from prostacyclin. Selexipag is hydrolyzed by carboxylesterase 1 to yield its active metabolite, which is approximately 37-fold as potent as selexipag.

What is Selexipag used for?

According to FDA labeling, Selexipag carries indications including: UPTRAVI is a prostacyclin receptor agonist indicated for the treatment of pulmonary arterial hypertension (PAH, WHO Group I): In adults to delay disease progression and reduce the risk of hospitalization for PAH. ( 1.1 ) In pediatric patients aged 2 years and older.. This is a reference summary of labeled uses, not medical advice or a treatment recommendation.

What class of drug is Selexipag?

Selexipag is classified as Platelet aggregation inhibitors excl. heparin, Prostacyclin Receptor Agonist, Cytochrome P450 2C8 Inhibitors, Prostacyclin Receptor Agonists, Decreased Blood Pressure, Pulmonary Arterial Vasodilation.

What are the brand names for Selexipag?

Selexipag is marketed under brand names including Uptravi.

What are the contraindications for Selexipag?

Selexipag labeling lists contraindications including: Hypersensitivity to the active substance or to any of the excipients. Concomitant use of strong inhibitors of CYP2C8 (e.g., gemfibrozil) [see Drug Interactions (7.1) and Clinical Pharmacology (12.3) ] .. Always consult the full prescribing information and a clinician.