Selexipag in SSc-associated pulmonary arterial hypertension: long-term real-world data from a multicentre Italian cohort

OBJECTIVES: Pulmonary arterial hypertension (PAH) is a life-threatening complication of SSc. Although selexipag is approved for SSc-PAH, available data are limited. We evaluated the long-term safety and clinical predictors associated with selexipag therapy in a multicentre Italian cohort of SSc-PAH patients. METHODS: We retrospectively analysed consecutive SSc patients with PAH diagnosed by right heart catheterization and treated with selexipag. Survival, treatment persistence, 1-year mortality risk (COMPERA 2.0) and predictors of clinical outcomes were assessed using appropriate statistical methods. RESULTS: Fifty-one SSc-PAH patients (94% female, median age 71 years) received selexipag for a median (interquartile range-IQR) duration of 22 (11-44) months. The estimated survival at 3 and 5 years from PAH diagnosis was 88% and 70%, respectively; a lower COMPERA 2.0 was the only significant protective factor. Right ventricular enlargement was associated with higher mortality risk (aOR 0.01, 95% CI 0.01-0.27), higher tricuspid annular plane systolic excursion with lower risk (aOR 1.35, 95% CI 1.04-1.75). After 12 months of treatment, the COMPERA 2.0 was improved in all patients, but a low risk of death was significantly more frequent in patients starting selexipag within 1 year from PAH onset (38% vs 8%, P < 0.01). Treatment persistence reached 84% at 12 months and baseline combination PAH therapy was associated with lower rates of selexipag discontinuation. CONCLUSIONS: Selexipag demonstrated a favourable long-term safety profile in SSc-PAH patients. A preserved right ventricular function represents a key determinant for survival, while early therapeutic combination strategies including selexipag are associated with more favourable COMPERA 2.0 mortality risk.