Uptravi (selexipag) / J&J, Nippon Shinyaku - LARVOL DELTA

Investigational randomized controlled trial of patients with inoperable chronic thromboembolic pulmonary hypertension treated with riociguat vs selexipag (ESC-WCC 2025) - "Riociguat and selexipag was significantly effective in treatment naive patients with inoperable CTEPH. Selecting the drug based on the characteristics of each drug could contribute to more favorable result in patients with CTEPH."

Clinical • Cardiovascular • Pulmonary Embolism

Comparison of triple oral and triple parenteral combination therapy in pulmonary arterial hypertension (ESC-WCC 2025) - "Background: The event-driven, phase 3, randomized, double-blind, placebo-controlled GRIPHON trial demonstrated that Selexipag reduces the risk of a composite end point of death or morbidity events in pulmonary arterial hypertension (PAH)... S was prescribed to the oldest and least severe PAH patients, while T and E to the youngest and most severe PAH patients and led to a stronger improvement of exercise capacity and haemodynamic profile."

Combination therapy • Cardiovascular

CTRP7 as a molecular biomarker for predicting responsiveness to pulmonary vasodilators: insights from human and animal studies in pulmonary arterial hypertension. (ESC-WCC 2025) - "CTRP7 was identified as a potential biomarker for predicting responsiveness to PGI2 analogs in PAH. PAH patients had higher plasma CTRP7 levels than non-PH individuals, and CTRP7 regulated PTGIR internalization through the IL-6-Rab5a axis, influencing the efficacy of selexipag. These findings suggest that CTRP7 may play a crucial role in guiding PAH treatment strategies."

Cardiovascular • IL6R • RAB5A

Efficacy and safety of selexipag based triple combination targeted drug therapy in chinese patients with pulmonary arterial hypertension (ESC-WCC 2025) - "Objective Selexipag, an oral prostacyclin receptor agonist, has been shown to be safe and effective for the treatment of pulmonary arterial hypertension (PAH). Conclusions Triple-targeted drug therapy containing selexipag can be safely and effectively used in Chinese PAH patients, significantly enhancing exercise capacity, improving right heart function and optimizing risk stratification. Selexipag-based initial triple therapy may potentially offer superior benefits in improving risk stratification compared to sequential add-on therapy for PAH patients."

Clinical • Cardiovascular

Could we maintain the initial efficacy of triple sequential combination therapies with selexipag in patients with pulmonary arterial hypertension : Insights from a single-centre study (ESC-WCC 2025) - "Our results seem to be consistent with efficacy and tolerability of selexipag-including triple sequential combinations in PAH. Baseline SPAHR, REVEAL 2.0, REVEAL lite 2.0 risk status, but not selexipag doses attained, seem to be associated with mortality. However, a trend for the attenuation in the efficacy after first year of selexipag should also be taken into consideration."

Clinical • Combination therapy • Cardiovascular

A real-world study on selexipag for pulmonary arterial hypertension: a single-center retrospective study (ESC-WCC 2025) - " We retrospectively investigated 97 consecutive patients with PAH who received selexipag between 2016 and 2022. In the real-world setting of patients with PAH, selexipag demonstrated improvements in hemodynamics, exercise capacity, and risk classification and did not add newly defined safety signals. These findings are supportive to the efficacy and tolerability of a unique oral PGI₂ receptor agonist selexipag for the favorable management of PAH."

Real-world • Real-world evidence • Retrospective data • Cardiovascular • Heart Failure

Characterization of the Population With Intermediate-Risk Status in Pulmonary Arterial Hypertension: Patients in a Latin American Country: The CAPRI Registry. (PubMed, Pulm Circ) - "The use of bosentan decreased from 36% (63/175) to 28% (46/154) (p < 0.001), while ambrisentan increased from 18% (31/175) to 30% (47/154), and macitentan increased from 25% (44/175) to 36% (57/154). The use of parenteral prostanoids and selexipag increased by 20% (4% vs. 24%) and 3% (1% vs. 4%) during clinical follow-up, respectively...At baseline, most patients were classified as intermediate-low-risk. However, during clinical follow-up, risk distribution changed, with a notable proportion shifting across different risk categories."

Journal • Cardiovascular • Hypertension • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

Evaluation of non-invasive low-risk criteria in patients with connective tissue disease-associated pulmonary arterial hypertension: A 12-months analysis from the INSPECTIO study. (PubMed, Vascul Pharmacol) - "CTD-PAH patients showed improvement in non-invasive risk criteria and stabilization of functional, echocardiographic, and hemodynamic parameters under macitentan and/or selexipag therapy. Despite the observational nature and small sample size, this real-world analysis supports the use of risk-based treatment strategies and close monitoring in this patient population."

Journal • Cardiovascular • Hypertension • Immunology • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases • Rheumatology • Scleroderma • Systemic Sclerosis

Real-world clinical outcomes according to individualised selexipag dose in pulmonary arterial hypertension (PAH) (ERS 2025) - No abstract available

Clinical • Clinical data • Real-world • Real-world evidence • Cardiovascular • Pulmonary Arterial Hypertension • Respiratory Diseases

Characteristics of Patients with Pulmonary Arterial Hypertension Receiving Selexipag in the SPHERE Registry by Race and Ethnicity. (PubMed, J Racial Ethn Health Disparities) - "Selexipag was prescribed as part of combination therapy for PAH regardless of differing demographics and clinical characteristics across racial/ethnic groups, with no differences in selexipag discontinuation, hospitalization, and survival."

Journal • Cardiovascular • Hypertension • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases • Rheumatology

Successful upfront combination therapy against pulmonary arterial hypertension associated with unilateral absence of the pulmonary artery: a case report. (PubMed, Eur Heart J Case Rep) - "Therefore, triple therapy with macitentan (10 mg), selexipag (0.4 mg), and riociguat (3 mg) was initiated...One year later, due to recurrent dyspnoea on light exertion, iloprost inhalation therapy was initiated. The patient has since progressed well, maintaining WHO class II during a 7-year follow-up period. Prostacyclin inhalation combined with triple therapy can be an effective treatment strategy for patients with UAPA-associated pulmonary arterial hypertension."

Journal • Acute Coronary Syndrome • Cardiovascular • Hypertension • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases • Sleep Apnea • Sleep Disorder

Reverse Bernheim Phenomenon - A True Enigma. (PubMed, Respir Med Case Rep) - "He was treated with ambrisentan, sildenafil and selexipag. The patient's PAH therapy was subsequently escalated with addition of intravenous treprostinil, which led to improvement of exercise capacity and resolution of syncopal episodes. A repeat stress echocardiogram revealed resolution of the acute exercise-induced RV dilatation and septal bulging without compressing the LVOT."

Journal • Cardiovascular • Congestive Heart Failure • Heart Failure • Hypertension • Hypotension • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

Prostacyclin Therapy as Adjunctive Treatment for Pulmonary Hypertension in Pediatric Heart Transplantation: A Case Report. (PubMed, Pediatr Transplant) - "This case highlights that aggressive medical management, including prostacyclin therapy, can render even high-risk pediatric patients with elevated pulmonary vascular resistance eligible for heart transplant and improve post-operative outcomes. It supports emerging evidence that elevated pulmonary vascular resistance, particularly when responsive to vasodilators, should not be viewed as an absolute contraindication to heart transplantation and emphasizes the importance of tailored, ongoing pulmonary hypertension management in children with congenital heart disease."

Journal • Cardiovascular • Congestive Heart Failure • Heart Failure • Hypertension • Pediatrics • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases • Transplantation

New horizons in systemic sclerosis treatment: advances and emerging therapies in 2025. (PubMed, RMD Open) - "While recent EULAR recommendations have updated the standard of care for SSc, the field is rapidly evolving with novel therapeutic strategies and precision medicine approaches.Traditional immunosuppressive therapies-including mycophenolate mofetil, cyclophosphamide and rituximab-remain essential for controlling skin and lung involvement while autologous haematopoietic stem cell transplantation offers a proven disease-modifying option for selected high-risk patients. Tocilizumab and nintedanib have established roles in lung preservation in SSc-associated interstitial lung disease (SSc-ILD). In pulmonary arterial hypertension (PAH), early combination therapy with endothelin receptor antagonists and phosphodiesterase-5 inhibitors, complemented by newer agents such as selexipag and riociguat, has improved survival and quality of life. Advances in gastrointestinal, renal and musculoskeletal management continue to evolve, with promising roles for intravenous immunoglobulin and..."

IO biomarker • Journal • Review • Bone Marrow Transplantation • Cardiovascular • Fibrosis • Gastrointestinal Disorder • Hypertension • Immunology • Interstitial Lung Disease • Musculoskeletal Diseases • Peptic Ulcer • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases • Rheumatology • Scleroderma • Systemic Sclerosis • Transplantation

INSPECTIO: A Study of Pulmonary Arterial Hypertension Participants Treated With Macitentan or Selexipag (clinicaltrials.gov) - P=N/A | N=372 | Completed | Sponsor: Janssen-Cilag S.p.A. | N=186 ➔ 372

Enrollment change • Cardiovascular • Hypertension • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

Efficacy and Safety of Selexipag Treatment in Connective Tissue Disease-Associated Pulmonary Arterial Hypertension with Concomitant Interstitial Lung Disease. (PubMed, Life (Basel)) - "While other systemic agents used in PAH have not shown as much success as inhaled treprostinil in treating PH-ILD, our case series highlights the potential role of selexipag in patients with concomitant CTD-PAH and ILD. Further investigation of selexipag in pure Group 3 PH-ILD patients is warranted."

Journal • Cardiovascular • Hypertension • Interstitial Lung Disease • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases • Rheumatology

LONG-TERM EFFECT OF SELEXIPAG IN SYSTEMIC SCLEROSIS-ASSOCIATED DIGITAL ULCERS: A CASE CONTROL, MULTICENTRE, OBSERVATIONAL STUDY (EULAR 2025) - "Our study shows that SSc patients treated with selexipag have a better sustained outcome of DUs compared to those treated with intravenous iloprost over 24 months. Furthermore, oral administration and good tolerability profile make selexipag a promising alternative option to standard therapy for SSc-related DUs, overcoming challenges such as difficult venous access, high hospitalization costs, and lost work productivity associated with IV administration. Prospective studies are needed to confirm a wider use of selexipag other than PAH."

Clinical • Observational data • Cardiovascular • Immunology • Pain • Pulmonary Arterial Hypertension • Respiratory Diseases • Rheumatology • Scleroderma • Systemic Sclerosis

CTRP7 as a molecular biomarker associating with responsiveness to pulmonary vasodilators: insights from human and animal studies in pulmonary arterial hypertension. (PubMed, Cardiovasc Res) - "In patients with PAH, RNA sequencing of PASMCs revealed elevated expression of CTRP7 among candidate biomarkers. Patients with PAH had higher plasma CTRP7 levels than those without PH. Mechanistically, CTRP7 regulated PTGIR internalization via the IL-6-Rab5a axis, influencing responsiveness to selexipag. Herein, CTRP7 emerged as a crucial biomarker associating with responsiveness to prostacyclin analogues, advancing the development of PAH treatment strategies."

Biomarker • Journal • Cardiovascular • Hypertension • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases • IL6R • RAB5A

REAL-LIFE EFFECTIVENESS OF SELEXIPAG TREATMENT IN SYSTEMIC SCLEROSIS ASSOCIATED PULMONARY ARTERIAL HYPERTENSION: LONG-TERM DATA FROM A MONOCENTRIC ITALIAN COHORT (EULAR 2025) - "Most frequently prescribed DMARD was mycophenolate mofetil (40%), followed by hydroxychloroquine (30%) and colchicine (20%). Selexipag treatment represents a valuable therapeutic option for SSc-PAH patients, with a reassuring long-term safety profile and encouraging survival data."

Clinical • Cardiovascular • Fibrosis • Hepatology • Immunology • Infectious Disease • Interstitial Lung Disease • Novel Coronavirus Disease • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases • Rheumatology • Scleroderma • Systemic Sclerosis

Safety and pharmacokinetics of ralinepag, a novel oral prostacyclin receptor agonist. (PubMed, JHLT Open) - "One received a single dose of ralinepag IR (30 µg) followed by single ascending doses of XR (60, 120, and 180 µg), and the other received single ascending doses of selexipag (200, 400, and 600 µg). Ralinepag XR has a half-life suitable for once-daily dosing. The reduced Cmax and delayed Tmax contributes to lower peak-to-trough fluctuations and may provide favorable effects for sustained efficacy and improved tolerability."

Journal • PK/PD data • Cardiovascular • Hypertension • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

Long-term effects of selexipag in systemic sclerosis-associated digital ulcers: a case control multicentre observational study. (PubMed, Rheumatology (Oxford)) - "This observational study suggests that selexipag may be strongly effective in treating DUs refractory to conventional drugs."

Journal • Observational data • Cardiovascular • Hypertension • Immunology • Pain • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases • Rheumatology • Scleroderma • Systemic Sclerosis

Maternal Pulmonary Arterial Hypertension Treated With IV Epoprostenol - A Case Report (ATS 2025) - "Her pre-gestation medications included Ambrisentan, Selexipag, and Sildenafil...The patient was then transitioned from IV Epoprostenol to inhaled Treprostinil via dry powder inhaler...IV Epoprostenol may be used adjunctively in the management of pregnant PAH patients and could improve peri and postpartum outcomes. This case adds to a growing literature base on this subject; the difficulties of performing a blinded, randomized clinical trial in this cohort are acknowledged."

Case report • Clinical • Cardiovascular • Pulmonary Arterial Hypertension • Respiratory Diseases

Efficacy and Safety of Selexipag in Connective Tissue Disease-Associated Pulmonary Arterial Hypertension With Concomitant Interstitial Lung Disease (ATS 2025) - "Pulmonary vasodilators have long been unsuccessfully studied in PH-ILD patients until the INCREASE trial demonstrated the effectiveness of inhaled Treprostinil in improving exercise capacity in this subgroup of Group 3 PH, highlighting the potential of the prostacyclin pathway to benefit these patients without worsening oxygenation. While other systemic agents used in treating PAH have not demonstrated a similar benefit as inhaled Treprostinil in treatment of PH-ILD, our study highlights the potential role of Selexipag in patients with concomitant CTD-PAH and parenchymal lung disease. Selexipag demonstrated statistically significant improvements in exercise capacity, as well as reductions in eRVSP; importantly, there was no evidence of worsening ventilation-perfusion mismatch in this cohort."

Clinical • Cardiovascular • Interstitial Lung Disease • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases • Rheumatology

Characteristics of Daily Walking Bouts as Valid and Reliable Indicators of Exercise Capacity in Pulmonary Arterial Hypertension: Insights From the Randomized Controlled Study With Selexipag (TRACE). (PubMed, Pulm Circ) - P4 | "In conclusion, actigraphy-derived metrics of patients' walking bouts correlate significantly with 6MWD, BDI and physical impacts of PAH, indicating the utility of actigraphy in facilitating continuous monitoring of exercise capacity in adult patients with PAH. ClinicalTrials.gov: NCT03078907; URL: clinicaltrials.gov."

Journal • Cardiovascular • Hypertension • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

Real-world Retrospective Cohort Study of Oral Selexipag in Methamphetamine-associated Pulmonary Arterial Hypertension: An Interim Analysis From TEAM PAH (ATS 2025) - "To our knowledge, this is the first cohort study describing oral selexipag dosing patterns in patients with meth-PAH, who constitute an increasing proportion of the PAH population in the United States. Most patients were titrated to their individualized dose using a site-specific multidisciplinary, nurse-driven protocol based on a tailored approach. As treatment with parenteral prostacyclins may be challenging in meth-PAH patients, our findings suggest that targeting the prostacyclin pathway with oral selexipag may be a viable treatment option for this at-risk population."

Real-world • Real-world evidence • Retrospective data • Cardiovascular • Hypertension • Obstructive Sleep Apnea • Pulmonary Arterial Hypertension • Respiratory Diseases • Sleep Disorder