Selexipag is indicated for the treatment of pulmonary arterial hypertension (PAH, WHO Group I) to delay disease progression and reduce the risk of hospitalization due to PAH.

Selexipag is a selective, non-prostanoid IP prostacyclin receptor agonist. It is a pale yellow crystalline powder that is practically insoluble in water. In solid form, selexipag is highly stable, non-hygroscopic, and not light sensitive. Selexipag is an oral prostacyclin receptor (IP receptor) agonist structurally different from prostacyclin. It is metabolized by carboxylesterase-1 to produce an active metabolite that is approximately 37 times more potent than the parent compound. Both selexipag and its active metabolite selectively act on the IP receptor compared to other prostanoid receptors (EP1–4, DP, FP, and TP).

The recommended starting dose is 200 mcg twice daily. Tolerability may be improved when taken with food. Increase the dose by 200 mcg twice daily at weekly intervals to the highest tolerated dose up to 1600 mcg twice daily. The maintenance dose is determined by tolerability.

Pediatric use: Safety and effectiveness in pediatric patients have not been established.

Geriatric use: No overall differences have been observed.

Hepatic impairment: For Moderate hepatic impairment patients starting dose is 200 mcg once daily, increase the dose by 200 mcg once daily at weekly intervals to the highest tolerated dose up to 1600 mcg. Avoid use of Selexipag in patients with severe hepatic impairment (Child-Pugh class C).

Moderate CYP2C8 inhibitors (e.g., deferasirox, teriflunomide) may increase exposure to the active metabolite of selexipag. CYP2C8 inducers (e.g., rifampicin) may decrease exposure to the active metabolite. Concomitant use may require adjustment of selexipag dose, including up to doubling of the dose in certain cases.

The most common adverse effects of selexipag include nausea, diarrhea, vomiting, headache, jaw pain, muscle pain, decreased appetite, and reduced red blood cell count.

There are no adequate and well-controlled studies of selexipag use in pregnant women. It is unknown whether selexipag is excreted in human breast milk. Therefore, either breastfeeding or treatment with selexipag should be discontinued in nursing mothers based on clinical judgment.

Selexipag should be discontinued if signs and symptoms of pulmonary veno-occlusive disease (PVOD) develop in a patient.

Isolated cases of overdose up to 3200 mcg have been reported, with mild transient nausea as the only observed effect. In case of overdose, supportive and symptomatic treatment should be provided.

Non-prostanoid IP prostacyclin receptor agonist

Keep in a dry place, protected from light and heat. Keep out of reach of children.