Purpose The selective oral IP receptor agonist selexipag is approved to delay disease progression and reduce the risk of hospitalization in patients with pulmonary arterial hypertension (PAH). SPHERE (SelexiPag: tHe usErs dRug rEgistry) is a US registry accruing data on real-world selexipag use. Here we report interim data on patients transitioning from a different PPA to selexipag. Methods Patients newly initiated on selexipag or previously initiated with a documented titration scheme are eligible for this ongoing study. Data are collected at routine visits (total follow-up: 18 months). Transitioned patients were defined as those taking a PPA for ≥30 days at selexipag initiation who had stopped the initial PPA ≤7 days before, or those continuing with the initial PPA at selexipag initiation. Assessments at the clinic visit closest to selexipag initiation were considered “baseline”. Results Of the first 250 enrolled patients (data cut-off: August 21, 2018), 56 (22%) had transitioned to selexipag: 8 (14.3%), 28 (50.0%), 3 (5.4%), and 17 (30.4%) from an oral, inhaled, subcutaneous, or intravenous PPAs, respectively. 65% completed their transition within 60 days. There were no clear differences in baseline characteristics or clinical course in patients who had transitioned from inhaled or oral PPAs vs parenteral PPAs. Transitioned and non-transitioned patients had similar baseline characteristics, except transitioned patients had a longer median time from PAH diagnosis to selexipag initiation (5.6 vs 3.1 years) and more had idiopathic PAH (64% vs 49%). The median time to maintenance selexipag dose was similar (8.1 vs 8.6 weeks) but the median maintenance dose was higher (1400 vs 1200 µg BID) for transitioned patients. Of patients who had baseline and 12-month data (transitioned, n=39; non-transitioned n=112), 10.3% and 9.8% of transitioned and non-transitioned patients had worsened FC, 69.2% and 74.1% had stable FC, and 20.5% and 16.1% had improved FC. With similar median time on selexipag (19.7 vs 17.8 months), 29% of transitioned and 34% of non-transitioned patients discontinued selexipag with 19.6% in each group discontinuing due to adverse events. Conclusion Baseline characteristics, clinical course, and selexipag tolerability were generally similar in transitioned vs non-transitioned patients. The selective oral IP receptor agonist selexipag is approved to delay disease progression and reduce the risk of hospitalization in patients with pulmonary arterial hypertension (PAH). SPHERE (SelexiPag: tHe usErs dRug rEgistry) is a US registry accruing data on real-world selexipag use. Here we report interim data on patients transitioning from a different PPA to selexipag. Patients newly initiated on selexipag or previously initiated with a documented titration scheme are eligible for this ongoing study. Data are collected at routine visits (total follow-up: 18 months). Transitioned patients were defined as those taking a PPA for ≥30 days at selexipag initiation who had stopped the initial PPA ≤7 days before, or those continuing with the initial PPA at selexipag initiation. Assessments at the clinic visit closest to selexipag initiation were considered “baseline”. Of the first 250 enrolled patients (data cut-off: August 21, 2018), 56 (22%) had transitioned to selexipag: 8 (14.3%), 28 (50.0%), 3 (5.4%), and 17 (30.4%) from an oral, inhaled, subcutaneous, or intravenous PPAs, respectively. 65% completed their transition within 60 days. There were no clear differences in baseline characteristics or clinical course in patients who had transitioned from inhaled or oral PPAs vs parenteral PPAs. Transitioned and non-transitioned patients had similar baseline characteristics, except transitioned patients had a longer median time from PAH diagnosis to selexipag initiation (5.6 vs 3.1 years) and more had idiopathic PAH (64% vs 49%). The median time to maintenance selexipag dose was similar (8.1 vs 8.6 weeks) but the median maintenance dose was higher (1400 vs 1200 µg BID) for transitioned patients. Of patients who had baseline and 12-month data (transitioned, n=39; non-transitioned n=112), 10.3% and 9.8% of transitioned and non-transitioned patients had worsened FC, 69.2% and 74.1% had stable FC, and 20.5% and 16.1% had improved FC. With similar median time on selexipag (19.7 vs 17.8 months), 29% of transitioned and 34% of non-transitioned patients discontinued selexipag with 19.6% in each group discontinuing due to adverse events. Baseline characteristics, clinical course, and selexipag tolerability were generally similar in transitioned vs non-transitioned patients.
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