{
  "abstract": "Introduction Systemic sclerosis (SSc) is associated with significant morbidity and mortality. Pulmonary arterial hypertension (PAH) represents a negative prognostic factor, with a 3-year survival of 50%. Selexipag, a selective prostacyclin receptor agonist, was approved in 2017 for the tratment of SSc-PAH, but few data on its use in a real-life setting are available. This study evaluated the long-term safety and effectiveness of Selexipag in a multicentric Italian cohort of SSc-PAH patients.Material and Methods Patients meeting the 2013 ACR/EULAR classification criteria for SSc, with PAH diagnosed at right heart catheterism (RHC), and treated with Selexipag, were retrospectively included. Data were analyzed with GraphPad Prism (v.9).Results Forty-four ssc-pah patients (93% female) from 12 italian referral centers were treated with selexipag for a median (interquartile range - iqr) of 19 (10-45) months ( table 1). The most common SSc skin subset was limited (79.5%), followed by sine-scleroderma (11.4%) and diffuse (9.1%). All patients had anti-nuclear antibodies (ANA), and 72.1% had anti-CENP antibodies. SSc-PAH survivors were significantly younger (p=0.02). Other clinical and laboratory characteristics were similar between groups. At the baseline 6-minutes walking distance test, SSc-PAH survivors performed significantly better than deceased patients (p=0.01). The estimated pulmonary artery pressure (PAP) (p=0.05) and tricuspid regurgitant velocity (p=0.03) at baseline echocardiography were significantly higher in deceased SSc-PAH patients. Similarly, the systolic PAP at RHC was significantly higher in deceased patients (p=0.03). The optimized median (IQR) Selexipag dose was 1600 (800-2400) microg/day and similar between groups. The median (IQR) timespan from PAH diagnosis to Selexipag start was 46 (16-175) months, with less than 30% of patients starting Selexipag within 2 years from PAH diagnosis. Reasons for Selexipag discontinuation included 5 (11.4%) adverse drug reactions (ADR), mainly diarrhea and headache, in the absence of life-threatening events, and one (2.3%) primary inefficacy. Selexipag persistency was 65.9% at 12 months and 40.9% at 24 months (figure 1). No significant differences were present in other treatments between groups. After a median (IQR) timespan of 9 (6-15) years, the survival rate was 52.3%. All patients received appropriate therapeutic adjustments, as 93.2% and 70.4% of them were on double and triple therapy for PAH at last follow-up, respectively.Conclusions Selexipag represents a valuable therapeutic option for SSc-PAH patients, with a reassuring long-term safety profile and encouraging survival data in real-life. Nonetheless, the time required to start the PAH triple combination therapy is still long. Shortening this timespan may further improve the prognosis of SSc-PAH patients.Abstract P.128 Table 1Comparison of baseline characteristics in alive and dead SSc-PAH patientsAbstract P.128 Figure 1Kaplan Meier curve of selexipag treatment persistency",
  "authors": [
    {
      "affiliations": [
        "Rheumatology Unit - Department of Precision and Regenerative Medicine, Jonian Area (DiPReMeJ), University of Bari ‘Al, Bari, Italy",
        "LUM ‘G. Degennaro’ - Department of Medicine and Surgery, Casamassima, Italy., Casamassima, Italy"
      ],
      "name": "Stefano Stano"
    },
    {
      "affiliations": [
        "Rheumatology Unit - Department of Precision and Regenerative Medicine, Jonian Area (DiPReMeJ), University of Bari ‘Al, Bari, Italy",
        "Rheumatology Unit - F. Miulli General Hospital, Department of Medicine and Surgery, LUM G. Degennaro, Casamassima, Italy"
      ],
      "name": "Fabio Cacciapaglia"
    },
    {
      "affiliations": [
        "Rheumatology Unit - Department of Precision and Regenerative Medicine, Jonian Area (DiPReMeJ), University of Bari ‘Al, Bari, Italy"
      ],
      "name": "Mariangela Nivuori"
    },
    {
      "affiliations": [
        "San Raffaele Scientific Institute and Vita-Salute San Raffaele University, (UnIRAR), Milano, Italy"
      ],
      "name": "Corrado Campochiaro"
    },
    {
      "affiliations": [
        "San Raffaele Scientific Institute and Vita-Salute San Raffaele University, (UnIRAR), Milano, Italy",
        "Scleroderma Clinic, Rheumatology Department, ASST G. Pini-CTO, Università degli Studi di Milano, Milano, Italy"
      ],
      "name": "Claudia Iannone"
    },
    {
      "affiliations": [
        "Department of Cardio-thoraco-vascular diseases, Fondazione IRCCS Ca’ Granda-Ospedale Maggiore Policlinico, Milano, Italy"
      ],
      "name": "Marco Vicenzi"
    },
    {
      "affiliations": [
        "Scleroderma Unit, Rheumatology and Clinical Immunology Unit, ASST Spedali Civili and University of Brescia, Brescia, Italy"
      ],
      "name": "Maria Grazia Lazzaroni"
    },
    {
      "affiliations": [
        "Rheumatology Unit, Department of Medical and Surgical Sciences, University of Foggia, Foggia, Italy"
      ],
      "name": "Cinzia Rotondo"
    },
    {
      "affiliations": [
        "Dipartimento di Scienze Cliniche Internistiche, Anestesiologiche e Cardiovascolari, Sapienza University of Rome, Roma, Italy"
      ],
      "name": "Marius Cadar"
    },
    {
      "affiliations": [
        "Rheumatology Unit, Medicine Unit, Casa Sollievo della Sofferenza-IRCCS-San Giovanni Rotondo, Foggia, Italy"
      ],
      "name": "Angelo De Cata"
    },
    {
      "affiliations": [
        "Division of Rheumatology, A.O.U. Policlinico-San Marco, Catania, Catania, Italy"
      ],
      "name": "Roberta Foti"
    },
    {
      "affiliations": [
        "Rheumatology, Allergology and Clinical Immunology, Department of Systems Medicine, University of Rome Tor Vergata, Roma, Italy"
      ],
      "name": "Maria Iacovantuono"
    },
    {
      "affiliations": [
        "Department of Rheumatology, University of Padua, Padova, Italy"
      ],
      "name": "Elisabetta Zanatta"
    },
    {
      "affiliations": [
        "Department of Biomedical Sciences, Humanitas University, Pieve Emanuele-Milan and IRCCS-Humanitas Research Hospital, Milano, Italy"
      ],
      "name": "Maria De Santis"
    },
    {
      "affiliations": [
        "Scleroderma Clinic, Rheumatology Department, ASST G. Pini-CTO, Università degli Studi di Milano, Milano, Italy"
      ],
      "name": "Nicoletta Del Papa"
    },
    {
      "affiliations": [
        "Scleroderma Clinic, Rheumatology Department, ASST G. Pini-CTO, Università degli Studi di Milano, Milano, Italy"
      ],
      "name": "Roberto Caporali"
    },
    {
      "affiliations": [
        "San Raffaele Scientific Institute and Vita-Salute San Raffaele University, (UnIRAR), Milano, Italy"
      ],
      "name": "arco Matucci-Cerinic"
    },
    {
      "affiliations": [
        "Rheumatology Unit - Department of Precision and Regenerative Medicine, Jonian Area (DiPReMeJ), University of Bari ‘Al, Bari, Italy"
      ],
      "name": "Florenzo Iannone"
    }
  ],
  "title": "P.128 Real-life effectiveness of Selexipag treatment in systemic sclerosis associated pulmonary arterial hypertension: long-term data from a multicentric Italian cohort",
  "uid": "d2b3564e-2da5-5057-99d3-92a5de1a5147"
}