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Introduction Pulmonary arterial hypertension (PAH) remains one of the main causes of mortality in Systemic Sclerosis (SSc) patients (Bruni et al. Eur J Intern Med. 2020). Macitentan, an orally active dual endothelin receptor antagonist, has been shown to reduce morbidity and mortality in a broad population of patients with pulmonary arterial hypertension (PAH), including those with PAH associated with systemic sclerosis (SSc-PAH) (Pulido et al. N Engl J Med. 2013). Moreover, a recent EUSTAR analysis identified the ESC/ERS four-strata model as an accurate tool for predicting long-term survival in patients with SSc-PAH (Bjørkekjær et al. Rheumatology 2025). This study aims to investigate the real-world persistency and long-term clinical outcomes of macitentan-treated SSc-PAH patients in the EUSTAR cohort.Material and Methods Adult SSc patients recorded in the EUSTAR database up to April 2023, fulfilling the 2013 ACR/EULAR criteria, on treatment with macitentan and with at least 6 months’ follow-up were included; main exclusion criterion was %pFVC<70. Patients who started macitentan within 6 months of first informative visit were incident users, and those on macitentan for >6 months at the first informative visit were prevalent users. Persistency of treatment was evaluated with Kaplan–Meier survival analysis, and different subgroups were compared with Log rank (Mantel-Cox) tests.Results Overall, 194 patients were included, with 119 having baseline data within 3 months of macitentan start. Characteristics at baseline (visit closest to macitentan initiation) for prevalent users (n=49), incident users (n=145) and the overall cohort are present in table 1. Most of the patients were low or intermediate-low risk (69%) and were in WHO class II or III (62%). Persistency of macitentan treatment (years, median [95%CI]) was greater in incident vs prevalent users (7.29 [6.69, 7.90] vs 5.98 [5.36, 6.59] vs; p=0019), in patients with baseline combination vs monotherapy (6.53 [5.77, 7.30] vs 5.33 [4.37, 6.28]; p=0.021) and in patients with cardiopulmonary comorbidities vs those without (6.62 [6.11, 7.14] vs 4.42 [3.36, 5.47]); p=0.025).After 12±3 months of treatment with macitentan, clinical effectiveness measures were stable/maintained or improved in 88% (esc/ers risk strata), 84% (who-fc) and 67% (6mwd) of patients (table 2).Conclusions The EUSTAR database offers a unique opportunity to evaluate real-life data on macitentan treatment patterns in SSc-PAH patients in a large multicenter international cohort. We found an improvement in clinical factors for PAH in SSc-PAH patients when treated with macitentan in the vast majority of the patients.Abstract P.129 Table 1Baseline characteristics of the populationAbstract P.129 Table 2Change in 4-risk strata score, 6MWD and WHO-FC from baseline to visit closest to 1-year follow-up after macitentan initiation in incident users