Systemic sclerosis & Raynaud's
Selexipag in SSc-associated pulmonary arterial hypertension: long-term real-world data from a multicentre Italian cohort
Authors' conclusion
Selexipag demonstrated a favourable long-term safety profile in SSc-PAH patients.
Study context
- Population
- Consecutive SSc patients with pulmonary arterial hypertension (PAH) diagnosed by right heart catheterization and treated with selexipag, multicentre Italian cohort (94% female, median age 71 years)94% female
- Participants
- 51 patients
- Outcome
- Long-term safety and clinical predictors associated with selexipag therapy: survival, treatment persistence, 1-year mortality risk (COMPERA 2.0)
Methodological detailsmulticentre, real-world, consecutive patients, Median (IQR) selexipag duration 22 (11-44) months
- Multicentre
- Real-world
- Consecutive patients
- Median (IQR) selexipag duration 22 (11-44) months
Main finding
As reported in the abstract
Estimated survival from PAH diagnosis · 3 and 5 years
- Estimated survival at 3 years from PAH diagnosis
- 88%
- Estimated survival at 5 years from PAH diagnosis
- 70%
- Right ventricular enlargement associated with higher mortality risk, aOR
- 0.01 (95% CI 0.01-0.27)
- Higher tricuspid annular plane systolic excursion associated with lower risk, aOR
- 1.35 (95% CI 1.04-1.75)
- Treatment persistence at 12 months
- 84%
Observational data: associations, not effects.
Further findings
Low COMPERA 2.0 risk of death, selexipag started within 1 year of PAH onset vs later · 12 months
- Low risk of death, selexipag within 1 year from PAH onset vs later (38% vs 8%, P < 0.01)
- COMPERA 2.0 improved in all patients
Selexipag discontinuation
- Baseline combination PAH therapy associated with lower rates of selexipag discontinuation
Original publication
Selexipag in SSc-associated pulmonary arterial hypertension: long-term real-world data from a multicentre Italian cohort. Rheumatology (Oxford). Published 7 September 2026. doi:10.
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