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Real-World Dosing and Titration of Selexipag in Patients With Pulmonary Arterial Hypertension in the SPHERE Registry (SelexiPag: tHe UsErs dRug rEgistry): Interim Analysis

Murali Chakinala

Nick H. Kim

Kelly Chin

Harrison Farber

Kristin Highland

Anna Hemnes

J. D. Carol

Michael Keating

Vallerie McLaughlin


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Conference: 2018 PHA International PH Conference & Scientific Sessions

Release Date: 06.28.2018

Presentation Type: Abstracts

File Download: Conference 2018_1028

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2018 International PH Conference and Scientific SeAbstract presented at the 2018 International PH Conference and Scientific Sessions in Orlando, Fla., June 28-July 1, 2018.


Selexipag is a selective oral IP prostacyclin receptor agonist for the treatment of pulmonary arterial hypertension (PAH) to delay disease progression and reduce the risk of hospitalization. The main objectives of the SPHERE registry are to describe: 1) dosing regimens and titration patterns; 2) disease characteristics; and 3) the clinical course of patients being treated with selexipag in real-world settings. The aim of the present report from SPHERE is to describe real-world dosing and titration patterns in patients newly initiated on selexipag.


SPHERE is a currently enrolling US-based, multicenter, prospective, real-world, observational registry initiated in November 2016. Patients are eligible if newly initiated on selexipag or being treated with selexipag at the time of enrollment and have a documented titration regimen. Safety data are collected on exposure of selexipag during the study. Maintenance dose is defined as the first dose post-titration that was maintained for 14 days or more without dose interruption and/or dose change.


This analysis (data cut-off October 2, 2017) reports data from the first 250 patients enrolled. The median time since PAH diagnosis was 4.2 years (range: 0.0–63.3). The median total duration of selexipag treatment was 11.6 months (range: 0.1–21.5), and the median on-study duration was 3.1 months (range: 0.1–12.1). Most patients (92%) had initiated selexipag prior to study enrollment. Patient characteristics are shown in the Table. Of the 234 patients with PAH medication data available, 65 (27.8%) were taking monotherapy without a prostacyclin, 108 (46.2%) were taking dual therapy without a prostacyclin, and 56 (23.9%) were on a regimen containing a prostacyclin at the time of selexipag initiation (See Table). The median time from selexipag initiation until a maintenance dose was reached was 8.1 weeks (interquartile range 6.0 to 12.1 weeks). The median selexipag maintenance dose was 1200 µg BID (interquartile range 800 µg BID to 1600 µg BID). Twenty-four patients (9.6%) discontinued selexipag because of adverse events with diarrhea being the most commonly occurring one (4/250, 1.6%).


In this first report of selexipag dosing in real-world settings, a median maintenance dose of 1200 µg BID was reached at a median of 8.1 weeks after initiation and no new safety signals were observed.