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Baseline and Demographic Data on the First 250 Patients From SPHERE (Uptravi® [SelexiPag]: tHe usErs dRug rEgistry)

Vallerie McLaughlin

Nick H. Kim

Kelly Chin

Kristin Highland

Anna Hemnes

Murali Chakinala

Michael Keating

Carol Zhao

J Colvin

Hap Farber


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Conference: 2018 PHA International PH Conference & Scientific Sessions

Release Date: 06.28.2018

Presentation Type: Abstracts

File Download: Conference 2018_1019

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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.


The selective oral IP receptor agonist selexipag is approved to delay disease progression and reduce the risk of hospitalization in pulmonary arterial hypertension (PAH) patients. SPHERE is a US drug registry of PAH patients designed to provide data on the real-world use of selexipag. The main objectives of SPHERE are to describe: 1) dosing regimens and titration schemes; 2) disease characteristics; and 3) the clinical course of patients being treated with selexipag. The aim of the present report from SPHERE is to describe the baseline characteristics of the first 250 patients enrolled.


Enrollment began in November 2016 with the goal of enrolling 500 patients. Patients previously or newly initiated on selexipag are eligible for the registry, however previously initiated patients must have a documented titration regimen. Data are collected at routine clinic visits; patients are to be followed for 18 months. The baseline information was analyzed by the times of diagnosis, selexipag initiation, and enrollment into the registry.


At selexipag initiation, the median age of the patient population was 61.0 years, the median duration of PAH was 4.2 years, the majority were women and white (both 73%). Approximately 50% were WHO functional class (FC) III while 26% were FC II and the median 6MWD was 314m. The most common comorbidities were: hypertension (56%), sleep apnea (37%), obesity (29%), diabetes (28%) and hypothyroidism (24%). Fifty percent of the patients had idiopathic PAH and 36% had associated PAH (29% connective tissue disease, 5% congenital heart disease and 2.9% portal pulmonary hypertension). Ninety-two percent had initiated selexipag prior to enrollment with a median duration of 7.1 months. At the time of selexipag initiation, 28% of patients were receiving monotherapy (ERA or PDE5i or sGC) and 47% were receiving dual therapy (ERA +PDE5i or sGC). Sixty patients (26%) were transitioned from a prostanoid to selexipag (24 patients from an IV/SQ prostanoid and 36 from an oral/inhaled prostanoid). The median maintenance selexipag dose was 1200mcg BID (IQR=800-1600). The total duration of exposure to selexipag was 11.6 months. No new safety profiles were observed than during the Phase III GRIPHON trial.


Of the first 250 patients enrolled in SPHERE most were WHO FC III women with iPAH or CTD. Nearly 47% of patients were receiving combination therapy with an ERA, PDE5i and/or sGC, 50% were receiving a maintenance dose of selexipag ≥1200mcg BID and 26% had been transitioned from a prostanoid.