{
  "abstract": "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",
  "authors": [
    {
      "affiliations": [
        "Rheumatology and Clinical Immunology, ASST Spedali Civili of Brescia, Italy",
        "Department of Clinical and Experimental Sciences, University of Brescia, Brescia, Italy"
      ],
      "name": "Maria-Grazia Lazzaroni"
    },
    {
      "affiliations": [
        "Unit of Immunology, Rheumatology, Allergy and Rare Diseases, IRCCS San Raffaele, Milan, Italy"
      ],
      "name": "Corrado Campochiaro"
    },
    {
      "affiliations": [
        "Rheumatology and Clinical Immunology, ASST Spedali Civili of Brescia, Italy",
        "Department of Clinical and Experimental Sciences, University of Brescia, Brescia, Italy"
      ],
      "name": "Paolo Airò"
    },
    {
      "affiliations": [
        "Department of Rheumatology, Endocrinology and Nephrology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University, Sapporo, Japan"
      ],
      "name": "Tatsuya Atsumi"
    },
    {
      "affiliations": [
        "Department of Internal Medicine and Rheumatology, Carol Davila University of Medicine and Pharmacy, Sf Maria Hospital, Bucharest, Romania"
      ],
      "name": "Andra Balanescu"
    },
    {
      "affiliations": [
        "Rheumatology Institute, Rambam Health Care Campus, Rappaport Faculty of Medicine, Technion, Haifa, Israel"
      ],
      "name": "Alexandra Balbir-Gurman"
    },
    {
      "affiliations": [
        "Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, and Department of Experimental Medicine, Division of Rheumatology, Azienda Ospedaliero Universitaria Careggi, University of Florence, Florence"
      ],
      "name": "Cosimo Bruni"
    },
    {
      "affiliations": [
        "Rheumatology Clinic, Department of Medical and Surgical Sciences, University of Foggia, Foggia, Italy"
      ],
      "name": "Francesco Paolo Cantatore"
    },
    {
      "affiliations": [
        "Rheumatology and Clinical Immunology, IRCCS-Humanitas Research Hospital, Rozzano",
        "Humanitas University, Pieve Emanuele, Milan, Milan, Italy"
      ],
      "name": "Maria De Santis"
    },
    {
      "affiliations": [
        "Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland"
      ],
      "name": "Oliver Distler"
    },
    {
      "affiliations": [
        "Division of Musculoskeletal and Dermatological Sciences, Faculty of Biology, Medicine and Health, The University of Manchester, Manchester, UK"
      ],
      "name": "Michael Hughes"
    },
    {
      "affiliations": [
        "Institute of Biometry and Clinical Epidemiology, Charité - Universitätsmedizin Berlin, Berlin, Germany"
      ],
      "name": "Dörte Huscher"
    },
    {
      "affiliations": [
        "Interdisciplinary Department of Medicine (DIM), Rheumatology Unit, Medical School, University of Bari, Bari, Italy"
      ],
      "name": "Florenzo Iannone"
    },
    {
      "affiliations": [
        "Department of Rheumatology, Radboud University Medical Center, Nijmegen, The Netherlands"
      ],
      "name": "Jacqueline Lemmers"
    },
    {
      "affiliations": [
        "Unità di Medicina Vascolare e Autoimmunità, CRIIS-Centro di riferimento interdisciplinare per la Sclerosi Sistemica, Sandro Pertini Hospital, Rome, Italy"
      ],
      "name": "Antonella Marcoccia"
    },
    {
      "affiliations": [
        "Department of Rheumatology & Immunology, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland"
      ],
      "name": "Britta Maurer"
    },
    {
      "affiliations": [
        "Rheumatology and Clinical Immunology, ASST Spedali Civili of Brescia, Italy",
        "Department of Clinical and Experimental Sciences, University of Brescia, Brescia, Italy"
      ],
      "name": "Liala Moschetti"
    },
    {
      "affiliations": [
        "Department of Autoimmune Diseases, Institut d’Investigacions Biomèdiques August Pi I Sunyer (IDIBAPS), Hospital Clínic, Barcelona, Spain"
      ],
      "name": "Ignasi Rodriguez-Pinto"
    },
    {
      "affiliations": [
        "Rheumatology Department, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal"
      ],
      "name": "Tania Santiago"
    },
    {
      "affiliations": [
        "Systemic Autoimmune Diseases Unit, Department of Internal Medicine, University Vall d’Hebron Hospital, Barcelona, Spain"
      ],
      "name": "Carmen-Pilar Simeón-Aznar"
    },
    {
      "affiliations": [
        "Department of Rheumatology & Clinical Immunology, University Medical Centre Utrecht, Utrecht, The Netherlands"
      ],
      "name": "Julia Spierings"
    },
    {
      "affiliations": [
        "Department of Rheumatology, University Hospital Basel, Basel, Switzerland"
      ],
      "name": "Ulrich Andreas Walker"
    },
    {
      "affiliations": [
        "Department of Medicine-DIMED, Division of Rheumatology, University of Padova, Padova, Italy"
      ],
      "name": "Elisabetta Zanatta"
    },
    {
      "affiliations": [
        "Unit of Immunology, Rheumatology, Allergy and Rare Diseases, IRCCS San Raffaele, Milan, Italy"
      ],
      "name": "Marco Matucci Cerinic"
    },
    {
      "affiliations": [
        "Rheumatology Department, CHU Bordeaux Hospital, Bordeaux, France"
      ],
      "name": "Marie-Elise Truchetet"
    },
    {
      "affiliations": [
        "Leeds Institute of Rheumatic and Musculoskeletal Medicine, LIRMM, Leeds, UK",
        "NIHR Leeds Biomedical Research Centre, Leeds, UK"
      ],
      "name": "Francesco Del Galdo"
    },
    {
      "affiliations": [
        "Department of Rheumatology, Radboud University Medical Center, Nijmegen, The Netherlands"
      ],
      "name": "Madelon C Vonk"
    }
  ],
  "title": "P.129 Macitentan for pulmonary arterial hypertension associated with systemic sclerosis: results from the European Scleroderma trials and research group (EUSTAR) database",
  "uid": "08623874-ac9e-5499-90c1-79884a5cb5d3"
}