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August 11, 2026European Respiratory JournalOpen Access

Selexipag for the treatment of connective tissue disease-associated pulmonary arterial hypertension

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Authors

SGSeán GaineKCKelly ChinGCGerry Coghlan

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Overview

Randomized trial evaluates selexipag's impact on morbidity/mortality in connective tissue disease patients, suggesting significant benefits.

Key Points

  • This study aims to evaluate the effectiveness of selexipag in reducing morbidity and mortality in patients with connective tissue disease-associated pulmonary arterial hypertension (PAH-CTD).
  • Characterized the GRIPHON study PAH-CTD subgroup of 334 patients, including those with systemic sclerosis and systemic lupus erythematosus.
  • Used Cox proportional hazard models to calculate hazard ratios for morbidity/mortality.
  • Evaluated treatment effects across different CTD subtypes and baseline therapies.
  • Selexipag reduced the risk of composite morbidity/mortality events by 41% (HR 0.59; 95% CI 0.41-0.85).
  • Treatment effects remained consistent across CTD subtypes with interaction p-values of 0.87 and 0.89.
  • Adverse events were mostly associated with prostacyclin, known for selexipag treatment.

Cite This Study

Gaine et al. (2017) studied this question.

synapsesocial.com/papers/6a7b7377078b29a6923ff709https://doi.org/10.1183/13993003.02493-2016
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Also Consider

Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context:

  1. 1Selexipag in patients with pulmonary arterial hypertension associated with connective tissue disease (PAH‐CTD): Real‐world experience from EXPOSURE2024 · 12 citations
  2. 2Early selexipag initiation and long-term outcomes: insights from randomised controlled trials in pulmonary arterial hypertension2022 · 19 citations
  3. 3Targeting the Prostacyclin Pathway with Selexipag in Patients with Pulmonary Arterial Hypertension Receiving Double Combination Therapy: Insights from the Randomized Controlled GRIPHON Study2018 · 84 citations
  4. 4Selexipag for the treatment of pulmonary arterial hypertension2020 · 12 citations
  5. 5A real-world study on selexipag for pulmonary arterial hypertension: a single-center retrospective study2025