Trial reportThe European respiratory journal2017
Selexipag for the treatment of connective tissue disease-associated pulmonary arterial hypertension.
Trial report in The European respiratory journal, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 63 papers, 5 of them syntheses that pooled 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.
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.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
63 citing papers in PubMed, 5 syntheses or guidelines pooled it.
- Management of inflammatory cardiopulmonary manifestations in systemic lupus erythematosus: a systematic review.Advances in rheumatology (London, England) · 2026Pooled it
- Management of pulmonary arterial hypertension in systemic sclerosis: from classical treatments to new horizons.European respiratory review : an official journal of the European Respiratory Society · 2026Pooled it
- Treatment of pulmonary arterial hypertension in patients with connective tissue diseases: a systematic review and meta-analysis.Internal and emergency medicine · 2024Pooled it
- Comparing the efficacy and safety of low, medium, and high dosages of selexipag for treating pulmonary hypertension: A systematic review and meta-analysis.Animal models and experimental medicine · 2024Pooled it
- Long-Term Outcomes in Patients With Connective Tissue Disease-Associated Pulmonary Arterial Hypertension in the Modern Treatment Era: Meta-Analyses of Randomized, Controlled Trials and Observational Registries.Arthritis & rheumatology (Hoboken, N.J.) · 2021Pooled it
- Temporarily switching from oral to intravenous selexipag in patients with pulmonary arterial hypertension: safety, tolerability, and pharmacokinetic results from an open-label, phase III study.Respiratory research · 2021Trial
- Effectiveness of PAH Specific Treatment Is Comparable Between PAH Associated With Connective Tissue Disease and Idiopathic PAH: Data From the Multicentre National Registry.Pulmonary circulation · 2026Article
- Real-World Experience with Selexipag in Patients with Pulmonary Arterial Hypertension: Treatment Patterns and Outcomes across Baseline Risk Strata (SPHERE and EXPOSURE Studies).Drugs - real world outcomes · 2026Article
- Towards an integrated and proactive management of pulmonary hypertension in systemic sclerosis: a practical approach for early diagnosis and optimal patient management.Rheumatology advances in practice · 2026Review
- Research advances in connective tissue disease-associated pulmonary arterial hypertension with an emphasis on the Chinese population.Frontiers in medicine · 2026Review
- Immunosuppressive Therapy for Pulmonary Arterial Hypertension Associated with Connective Tissue Diseases: Insights from Japanese Registry.Advances in therapy · 2025Article
- Low DLCO Can Provide Insights into Treatment Response in PAH Patients Irrespective of the Reason for Its Decrease.Life (Basel, Switzerland) · 2025Article
- Prostaglandin E1 restores endothelial progenitor cell function in systemic sclerosis.Rheumatology (Oxford, England) · 2025Article
- Selexipag in patients with pulmonary arterial hypertension associated with connective tissue disease (PAH-CTD): Real-world experience from EXPOSURE.Pulmonary circulation · 2024Article
- Macitentan in Pulmonary Arterial Hypertension Associated with Connective Tissue Disease (CTD-PAH): Real-World Evidence from the Combined OPUS/OrPHeUS Dataset.Cardiology and therapy · 2024Article
- Systemic Sclerosis-Associated Pulmonary Arterial Hypertension: From Bedside to Bench and Back Again.International journal of molecular sciences · 2024Review
- Pharmacological Agents and Potential New Therapies in Pulmonary Arterial Hypertension.Current vascular pharmacology · 2024Review
- Real-World Treatment Patterns Among Patients with Connective Tissue Disorder-Related Pulmonary Arterial Hypertension in the United States: A Retrospective Claims-Based Analysis.Advances in therapy · 2023Article
- Connective tissue disease-associated pulmonary hypertension: A comprehensive review.Pulmonary circulation · 2023Review
- Recent Advances in the Treatment of Pulmonary Arterial Hypertension Associated with Connective Tissue Diseases.Pharmaceuticals (Basel, Switzerland) · 2023Review
3 more citing papers are in PubMed but not listed here.
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
15 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Patients with connective tissue disease-associated pulmonary arterial hypertension (PAH-CTD) have a poor prognosis compared with other aetiologies. The underlying CTD can influence treatment response and outcomes. We characterised the GRIPHON study PAH-CTD subgroup and evaluated response to selexipag.Of 334 patients with PAH-CTD, PAH was associated with systemic sclerosis (PAH-SSc) in 170, systemic lupus erythematosus (PAH-SLE) in 82 and mixed CTD/CTD-other in 82. For the primary composite endpoint of morbidity/mortality, hazard ratios (HR) and 95% CI were calculated using Cox proportional hazard models.Compared with the overall GRIPHON population, the CTD subgroup was slightly older with a greater proportion of females and shorter time since diagnosis. Patients with PAH-SSc appeared to be more impaired at baseline, with a more progressive disease course. The converse was observed for PAH-SLE. Selexipag reduced the risk of composite morbidity/mortality events in patients with PAH-CTD by 41% (HR 0.59; 95% CI 0.41-0.85). Treatment effect was consistent irrespective of baseline PAH therapy or CTD subtype (interaction p=0.87 and 0.89, respectively). Adverse events were predominately prostacyclin-related and known for selexipag treatment.GRIPHON has allowed the comprehensive characterisation of patients with PAH-CTD. Selexipag delayed progression of PAH and was well-tolerated among PAH-CTD patients, including those with PAH-SSc and PAH-SLE.
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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.