Evidence map›Paper›PMID 41525027›Full record

ArticleDrugs - real world outcomes2026

Real-World Experience with Selexipag in Patients with Pulmonary Arterial Hypertension: Treatment Patterns and Outcomes across Baseline Risk Strata (SPHERE and EXPOSURE Studies).

Pilar Escribano Subias, Murali M Chakinala, Kelly M Chin, Eva-Maria Didden, Gurinderpal Doad, Harrison W Farber, Sean Gaine, Anna R Hemnes, Kristin B Highland, Tobias J Lange and 7 more

Registry-linked trialAbstract read
In one paragraph

Article in Drugs - real world outcomes, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT03278002 (Uptravi®), which is not on this map. Not yet cited in PubMed.

0numbers the graph read from it
0cells of the map it votes in
0citing papers in PubMed
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1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

NCT03278002 completednot on this map

Uptravi® (SelexiPag): tHe usErs dRug rEgistry

Typeobservational_patient_registrySponsorActelionRan2016 to 2021Enrolled800ConditionsPulmonary Arterial Hypertension
3 · Its place in the literature

Who cites it

0 citing papers in PubMed.

No citing paper in PubMed yet.

4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

17 authors.

Pilar Escribano SubiasDepartment of Cardiology, CIBERCV, Instituto de Investigación del Hospital Universitario 12 de Octubre, ERN-LUNG, Universidad Complutense, Madrid, Spain. pilar.escribano.subias@outlook.com.
Murali M ChakinalaWashington University School of Medicine, St. Louis, MO, USA.
Kelly M ChinUniversity of Texas, Southwestern Medical Center, Dallas, TX, USA.
Eva-Maria DiddenJohnson & Johnson, Allschwil, Switzerland.
Gurinderpal DoadJohnson & Johnson, Titusville, NJ, USA.
Harrison W FarberTufts Medical Center, Boston, MA, USA.
Sean GaineNational Pulmonary Hypertension Unit, Mater Misericordiae University Hospital, Dublin, Ireland.
Anna R HemnesVanderbilt University Medical Center, Nashville, TN, USA.
Kristin B HighlandCleveland Clinic, Cleveland, OH, USA.
Tobias J LangeDepartment of Pulmonology, Bad Reichenhall Hospital, Bad Reichenhall, Germany.
Vallerie McLaughlinUniversity of Michigan Medical Center, Ann Arbor, MI, USA.
Audrey MullerJohnson & Johnson, Allschwil, Switzerland.
Mohammad RahmanJohnson & Johnson, Titusville, NJ, USA.
Tatiana RemeňováJohnson & Johnson, Allschwil, Switzerland.
Stefan SöderbergDepartment of Public Health and Clinical Medicine, Cardiology and Heart Centre, Umeå University, Umeå, Sweden.
Apoorva VenkateshJohnson & Johnson, Allschwil, Switzerland.
Nick H KimUniversity of California San Diego, La Jolla, CA, USA.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundTreatment strategies for pulmonary arterial hypertension (PAH) depend on patients' 1-year mortality risk; however, real-world practices and outcomes remain inadequately described.

objectiveWe aimed to investigate real-world experience with selexipag, an oral selective prostacyclin I2 receptor agonist used to treat PAH.

methodsThis analysis of two large, prospective, observational studies: SPHERE (NCT03278002; USA; completed) and EXPOSURE (EUPAS19085; Europe and Canada; ongoing, data cutoff November 2021) included adults newly initiating selexipag with follow-up available.

resultsOf 1366 selexipag users, 894 met eligibility criteria. At selexipag initiation, the median (Q1-Q3) age was 61 (48-70) years and the time since diagnosis was 2.7 (0.8-7.4) years. Most patients initiated selexipag within triple oral combination therapy (67%) with WHO-FC III symptoms (61%). During a median 10.8 (Q1-Q3: 3.3-18.6) months of selexipag exposure, 388 (43%) patients discontinued selexipag, mostly as a result of tolerability (n = 173, 19%) and death (n = 69, 8%). The median individualised dose was 800 μg twice-daily (n = 742). One-year mortality risk at selexipag initiation (assessable for 591 patients using a four-strata method): 22% were low-risk, 40% intermediate-low, 29% intermediate-high, and 9% high-risk. From low to high risk: 86%, 73%, 57% and 45% were free from all-cause hospitalisation at 1 year, and 1-year survival was 100%, 96%, 91%, and 59%, respectively.

conclusionsDespite current guidelines, over half of patients started selexipag almost 3 years after diagnosis, and 38% were already intermediate-high or high risk of 1-year mortality. Lower hospitalisation rates and better survival were observed for selexipag-treated patients in the low and intermediate-low risk groups versus higher risk groups.

Identifiers

PMID41525027
PMCPMC13003052

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Registered trials

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.