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A Retrospective Database Analysis

Comparative Effectiveness of UPTRAVI and Oral Treprostinil on Hospitalization in Patients With PH in the US1

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Studied in a Broad Range of Patients With PAH2

SPHERE (SelexiPag: tHe usErs dRug rEgistry) is the largest real-world registry of a prostacyclin pathway agent to date in the US2-5*

Study Design2

Patients aged ≥18 years were enrolled from November 2016 to March 2020 and followed for up to 18 months. UPTRAVI was initiated by the treating physician per routine clinical practice.† There were no study-mandated visits or procedures. Data were collected at enrollment and every 3 months thereafter via electronic case report. AEs were collected from enrollment to the last UPTRAVI dose. During the titration phase, AEs associated with the mode of action of UPTRAVI were collected only if they were defined as serious, led to discontinuation of UPTRAVI, or reflected an unusual pattern of severity according to investigator judgment.2

Study Limitations and Considerations2

  • The main limitations of this study are related to its observational nature, including: the potential bias introduced by including previously initiated patients for whom no data were collected between treatment initiation and study enrollment, incomplete records reflective of clinical practice can impact interpretation of findings, and the data analyses reported are descriptive only. No clinical conclusions can be drawn due to these limitations of this study
  • As a drug registry (rather than a disease registry), SPHERE recruited only patients receiving UPTRAVI, who typically take UPTRAVI as part of combination therapy. This introduced bias in favor of combination therapy recipients
  • The following may contain data, conclusions, and recommendations that do not conform to the US FDA-approved labeling for the product discussed herein. UPTRAVI should be used only as specified in the Prescribing Information for UPTRAVI. Due to the real-world nature of the study, patients receiving UPTRAVI who had PH but not PAH also participated; however, for the present analysis, we have focused on patients with PAH. Please see the full Prescribing Information for UPTRAVI for adequate directions for use for the approved indication
US-based, multicenter, prospective, real-world, observational drug registry enrolling patients actively treated with UPTRAVI (selexipag) and followed for up to 18 months.

759 Patients

Newly
initiated‡: 387

Previously initiated§: 372

Median age at UPTRAVI initiation: 
61 years

Female: 76.5%

2.7 years

Median time from PAH diagnosis to initiation of UPTRAVI

57.3%

Intermediate or high risk at baseline per REVEAL 2.0

80.5%

WHO FC II or III

SPHERE Registry Risk Status & Functional Class

Changes in Risk Status and Functional Class

Change in REVEAL 2.0 risk status from baseline among all patients with PAH at 18 months2
Change in FC from baseline among all patients with PAH at 18 months2
SPHERE Registry Survival

Overall Survival Rate in Patients With PAH 

Survival probability over the study period among all patients with PAH2

These uncontrolled observations do not allow comparison with a control group not given UPTRAVI and cannot be used to determine the effect of UPTRAVI on mortality.

SPHERE Registry Safety

Safety in the SPHERE Registry

Overview of AEs in the PAH Population
Adverse
Event		All patients
N=759
Any AE543 (71.5%)
Serious AE278 (36.6%)
AE leading to death54 (7.1%)
AE leading to hospitalization262 (34.5%)
AE leading to discontinuation188 (24.8%)
Related to UPTRAVI53 (7.0%)
Headache23 (3.0%)
Diarrhea11 (1.4%)
Myalgia12 (1.6%)
Nausea12 (1.6%)
Arthralgia6 (0.8%)
Pain in jaw4 (0.5%)
Adverse
Event		All patients
N=759
Related to PAH
progression 		107 (14.1%)
Pulmonary hypertension18 (2.4%)
Dyspnea15 (2.0%)
Right ventricular failure15 (2.0%)
Pulmonary arterial
hypertension		13 (1.7%)
Acute respiratory failure10 (1.3%)
Respiratory failure6 (0.8%)

Start your PAH patients on UPTRAVI

EXPLORE DOSING

*Based on published data as of January 7, 2025.

†Newly initiated=patients who started UPTRAVI ≤60 days before enrollment.

‡Previously initiated=patients who started UPTRAVI >60 days before enrollment.

§Patients were excluded if they had previously received UPTRAVI in a clinical trial, previously discontinued UPTRAVI for any reason before enrollment, or participated in a blinded clinical trial or trial of any unapproved drug.2

||By 18 months, 22.0% of patients with PAH had discontinued UPTRAVI (selexipag) due to AEs (32.0% of the newly initiated group; 11.9% of the previously initiated group).2

AE=adverse event; ERA=endothelin receptor antagonist; FC=Functional Class; FDA=US Food and Drug Administration; IPAH=idiopathic PAH; PAH=pulmonary arterial hypertension; PAH-CHD=PAH associated with congenital heart disease with repaired shunts; PAH-CTD=PAH associated with CTD; PDE5i=phosphodiesterase type-5 inhibitor; PGI2=prostacyclin; REVEAL=Registry to EValuate EArly and Long-term PAH Disease Management; sGCs=soluble guanylate cyclase stimulator; WHO=World Health Organization.

References: 1. UPTRAVI (selexipag) full Prescribing Information. Actelion Pharmaceuticals US, Inc. 2. McLaughlin V, Farber HW, Highland KB, et al. Disease characteristics, treatments, and outcomes of patients with pulmonary arterial hypertension treated with UPTRAVI in real-world settings from the SPHERE registry (SelexiPag: tHe usErs dRug rEgistry). J Heart Lung Transplant. 2024;43(2):272-283. 3. Lachant D, Minkin R, Swisher J, et al. Safety and efficacy of transitioning from selexipag to oral treprostinil in pulmonary arterial hypertension: findings from the ADAPT registry. Pulm Pharmacol Ther. 2023;82:102232. doi:10.1016/j.pupt.2023.102232 4. Frantz RP, Schilz RJ, Chakinala MM, et al. Hospitalization and survival in patients using epoprostenol for injection in the PROSPECT observational study. Chest. 2015;147(2):484-494. doi:10.1378/chest.14-1004 5. Evaluate Real-World Use of Next Generation Infusion Pumps to Administer Remodulin (EVOLVE). Clinicaltrials.gov identifier: NCT05060315. Updated January 16, 2025. Accessed April 15, 2026. https://clinicaltrials.gov/study/NCT05060315 6. Data on file. Actelion Pharmaceuticals US, Inc. SPHERE Adverse Events in Patients With PAH. February 9, 2024.