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

Authors

Stefano Stano, Claudia Iannone, Corrado Campochiaro, Marco Vicenzi, Maria Grazia Lazzaroni, Cinzia Rotondo, Marius Cadar, Angelo De Cata, Roberta Foti, Maria Iacovantuono, Elisabetta Zanatta, Francesca Motta, Antonella Grimaldi, Stefania Marazia, Eugenio Capparelli, Fabrizio Fortunato, Giuseppe Galgano, Andrea Cito, Ilaria Cavazzana, Nicoletta Del Papa, Roberto Caporali, Marco Matucci-Cerinic, Fabio Cacciapaglia, Florenzo Iannone

Published in

Rheumatology (Oxford, England). Sep 07, 2026. Epub Sep 07, 2026.

Abstract

Pulmonary arterial hypertension (PAH) is a life-threatening complication of systemic sclerosis (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.
We retrospectively analysed consecutive SSc patients with PAH diagnosed by right heart catheterization and treated with selexipag. Survival, treatment persistence, one-year mortality risk (COMPERA 2.0) and predictors of clinical outcomes were assessed using appropriate statistical methods.
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 one 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.
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.

PMID:
42704673
Bibliographic data and abstract were imported from PubMed on 08 Sep 2026.

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