Fabio Cacciapaglia, Rossella De Angelis, Clodoveo Ferri, Gianluigi Bajocchi, Silvia Bellando-Randone, Cosimo Bruni, Martina Orlandi, Marco Fornaro, Edoardo Cipolletta, Giovanni Zanframundo, Roberta Foti, Giovanna Cuomo, Alarico Ariani, Edoardo Rosato, Gemma Lepri, Francesco Girelli, Elisabetta Zanatta, Silvia Laura Bosello, Ilaria Cavazzana, Francesca Ingegnoli, Maria De Santis, Giuseppe Murdaca, Giuseppina Abignano, Pettiti Giorgio, Alessandra Della Rossa, Maurizio Caminiti, Annamaria Iuliano, Giovanni Ciano, Lorenzo Beretta, Gianluca Bagnato, Ennio Lubrano, Ilenia De Andres, Alessandro Giollo, Marta Saracco, Cecilia Agnes, Corrado Campochiaro, Federica Lumetti, Amelia Spinella, Luca Magnani, Giacomo De Luca, Veronica Codullo, Elisa Visalli, Carlo Iandoli, Antonietta Gigante, Greta Pellegrino, Franco Cozzi, Maria Grazia Lazzaroni, Elena Generali, Gianna Mennillo, Simone Barsotti, Giuseppa Pagano-Mariano, Federica Furini, Licia Vultaggio, Simone Parisi, Clara Lisa Peroni, Gerolamo Bianchi, Enrico Fusaro, Gian Domenico Sebastiani, Marcello Govoni, Salvatore D’Angelo, Erika Pigatto, Franco Franceschini, Serena Guiducci, Lorenzo Dagna, Andrea Doria, Dilia Giuggioli, Valeria Riccieri, Carlo Salvarani, Marco Matucci-Cerinic and Florenzo Iannone on behalf of SPRING-SIR (Systemic Sclerosis PRogression INvestiGation group of the Italian Society for Rheumatology)
Multiple Institutions
Italy
Journal of Rheumatology
J Rheumatol 2025;
DOI: 10.3899/jrheum.2024-0750
Abstract
Objective: Bosentan (BOS) is approved for treating pulmonary arterial hypertension (PAH) and preventing digital ulcers (DU) in systemic sclerosis (SSc). Our study aimed to evaluate whether BOS prescribed for DU could reduce the incidence of PAH in a large SSc cohort from the SPRING registry.
Methods: Patients with SSc from the SPRING registry, meeting ACR/EULAR 2013 classification criteria with data on PAH onset, DU status, BOS exposure, and at least a one-year follow-up between 2015 and 2020, and no known PAH at baseline were included. PAH was diagnosed with right heart catheterization during the follow-up, and its incidence rate (IR) was calculated. Kaplan-Meier curves were determined, and multivariate regression identified PAH risk factors.
Results: Among 727 eligible patients with SSc, followed for a median of 2.0 years, 54 (7.4%) developed PAH [IR 3.71 per 100 patients/years]. Patients with DU who were never exposed to BOS had a higher incidence of PAH [IR 4.90 per 100 patients/years] compared to those exposed to BOS, whose rates matched those without DU and who were never exposed to BOS. Risk factors independently associated with PAH development included DU (HR 1.85), age (HR 1.05), modified Rodnan Skin Score (mRSS) >4 (HR 2.07), ILD (HR 2.29), and acetylsalicylic acid treatment (HR 1.78).
Conclusion: In our cohort, DU were confirmed as a leading risk factor for PAH development, and BOS use for DU prevention may reduce this risk. Only patients with DU who were not on BOS had an increased PAH incidence.
Category
Class I. Pulmonary Hypertension Associated with Connective Tissue Disease
Medical Therapy. Efficacy or Lack of Efficacy
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
Article Access
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