Uptravi (selexipag)
An approved treatment for Pulmonary Arterial Hypertension.
The same compound appears under different names depending on the context. Here is how to identify Selexipag wherever you encounter it, plus the key facts at a glance.
- Generic name
- Selexipag
- Brand name
- Uptravi
- Development codes
- ACT-293987, NS-304
- Drug class
- Prostacyclin receptor agonist
- Manufacturer
- Janssen (Johnson & Johnson)
- How it's taken
- Available as oral tablets (twice daily) and IV formulation (approved 2021 for patients temporarily unable to take oral).
The first oral prostacyclin pathway agent that selectively targets the IP receptor. Reduces the risk of disease progression and hospitalization in PAH when added to existing treatment.
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Where Selexipag fits
Oral prostacyclin pathway therapy for PAH, often used in combination with endothelin receptor antagonists and/or PDE5 inhibitors as part of a multi-drug approach.
How Selexipag works
Selexipag selectively activates the IP prostacyclin receptor on pulmonary artery smooth muscle cells, causing vasodilation. Unlike treprostinil, which acts on multiple prostanoid receptors, selexipag is specific to the IP receptor. This selectivity may reduce some side effects while maintaining the benefit of prostacyclin pathway activation.
Mechanism: Selective IP prostacyclin receptor agonist that dilates pulmonary arteries
Side effects and safety
Common side effects include headache, diarrhea, jaw pain, nausea, muscle pain, vomiting, flushing, and extremity pain. These are typical prostacyclin-class effects and often improve over time. Dose titration over several weeks helps manage tolerability. If signs of fluid in the lungs (pulmonary edema) appear, your doctor should check for pulmonary veno-occlusive disease, and Uptravi should be stopped if that is confirmed. Do not take Uptravi with gemfibrozil or other strong CYP2C8 inhibitors.
This is not a complete list of side effects. Talk to your doctor or pharmacist about what to expect and when to seek medical attention.
Taking Selexipag
Available as oral tablets (twice daily) and IV formulation (approved 2021 for patients temporarily unable to take oral). In adults, the dose is individually titrated from 200 mcg twice daily up to 1600 mcg twice daily based on tolerability. Since May 2026 tablets are also approved for children 2 and older, with doses based on body weight.
Availability and cost
Generic versions may be available at a lower cost. Ask your pharmacist.
First oral selective prostacyclin IP receptor agonist, distinct from prostacyclin analogs. Oral convenience without the infusion or inhalation requirements of traditional prostacyclins.
Help paying for Uptravi
Pick your insurance to see which help fits. Drugmaker copay cards can't be used with Medicare, Medicaid or TRICARE; charity funds are the usual route there.
- Copay help
Eligible commercially insured patients pay $5 per fill; yearly maximum shared across J&J oral PAH medicines. Not valid with Medicare, Medicaid or other government programs.
For: private insurance · source - Free medicine program
Uninsured patients, or those whose insurance does not fully meet their needs, may get the medicine free for up to one year if they meet income and eligibility rules.
For: no insurance, underinsured, private insurance, Medicare, Medicaid · source - Insurance and case manager help
Care Coordinators explain insurance coverage and out-of-pocket costs and identify cost support options.
The official page does not say who qualifies. Ask the program. · source
Good to know: Savings are through the J&J withMe Oral PAH Savings Program. Free-drug terms at PatientAssistanceInfoPH.com.
- From a charity · HealthWell FoundationPulmonary Hypertension (Medicare Access) fundOpen
Pays for: Copays, premiums or other treatment costs. Medicare patients only.
- From a charity · TotalAssist (formerly PAN Foundation)Pulmonary Hypertension fundOpen
Pays for: Out-of-pocket costs for approved medications, up to $9,500 per year. Requires health insurance (any kind).
- From a charity · The Assistance FundPulmonary Hypertension fundWaitlist
Pays for: Copays, coinsurance, deductibles and other health-related expenses.
The foundation says: “WAITLIST — Accepting Waitlist Patients. TAF is currently accepting requests to join the enrollment waitlist for this program. Waitlists a…”
Clinical trial results
GRIPHON Phase 3 trial (1,156 patients, largest PAH trial ever at the time) showed 40% reduction in disease progression or hospitalization versus placebo when added to background therapy.
Development history
Approved by FDA in December 2015 based on the landmark GRIPHON trial. IV formulation added in 2021. Represented the first oral selective IP receptor agonist, offering prostacyclin pathway benefits without a pump or nebulizer.
Explore Pulmonary Arterial Hypertension trials
Other Pulmonary Arterial Hypertension treatments
Common questions about Selexipag
▸What is Selexipag (Uptravi)?
The first oral prostacyclin pathway agent that selectively targets the IP receptor. Reduces the risk of disease progression and hospitalization in PAH when added to existing treatment.
▸How does Selexipag work?
Selexipag selectively activates the IP prostacyclin receptor on pulmonary artery smooth muscle cells, causing vasodilation. Unlike treprostinil, which acts on multiple prostanoid receptors, selexipag is specific to the IP receptor. This selectivity may reduce some side effects while maintaining the benefit of prostacyclin pathway activation.
▸What are the side effects of Selexipag?
Common side effects include headache, diarrhea, jaw pain, nausea, muscle pain, vomiting, flushing, and extremity pain. These are typical prostacyclin-class effects and often improve over time. Dose titration over several weeks helps manage tolerability. If signs of fluid in the lungs (pulmonary edema) appear, your doctor should check for pulmonary veno-occlusive disease, and Uptravi should be stopped if that is confirmed. Do not take Uptravi with gemfibrozil or other strong CYP2C8 inhibitors.
▸How is Selexipag taken?
Available as oral tablets (twice daily) and IV formulation (approved 2021 for patients temporarily unable to take oral). In adults, the dose is individually titrated from 200 mcg twice daily up to 1600 mcg twice daily based on tolerability. Since May 2026 tablets are also approved for children 2 and older, with doses based on body weight.
▸Is Selexipag FDA approved?
Yes, Selexipag (Uptravi) is FDA approved (2015) for the treatment of Pulmonary Arterial Hypertension.
▸How does selexipag differ from treprostinil?
Both target the prostacyclin pathway but through different mechanisms. Treprostinil is a prostacyclin analog that directly activates multiple prostanoid receptors. Selexipag is a selective IP prostacyclin receptor agonist that targets only the IP receptor. This selectivity may provide a different side effect profile. Selexipag is taken orally twice daily, making it far more convenient than treprostinil infusions, though the inhaled and subcutaneous treprostinil formulations may deliver more potent prostacyclin pathway activation to the lungs.
▸What did the GRIPHON trial show?
GRIPHON was one of the largest PAH trials ever conducted, enrolling 1,156 patients. Selexipag reduced the composite endpoint of morbidity/mortality events (including disease progression, hospitalization, and death) by 40% compared to placebo when added to background PAH therapy. This was the first trial to show that adding an oral prostacyclin pathway agent to existing combination therapy could improve outcomes.
▸Why is dose titration important with selexipag?
Selexipag is started at a low dose (200 mcg twice daily) and increased weekly by 200 mcg increments until the highest tolerated dose is reached, up to 1600 mcg twice daily. This gradual titration is necessary because prostacyclin pathway side effects (headache, jaw pain, diarrhea, nausea, flushing) are dose-dependent and tend to be most pronounced during dose increases. Patients who titrate slowly and manage side effects tend to reach higher maintenance doses and may experience greater benefit.
▸Is there an IV formulation of selexipag?
Yes. An IV formulation of selexipag was approved in 2021 for patients who are temporarily unable to take the oral tablets, such as during hospitalization or perioperative periods. The IV form bridges patients through situations where oral medications cannot be taken, preventing gaps in prostacyclin pathway therapy.