{
  "abstract": "Introduction Interactions between vascular dysfunction, aberrant immune response, and inflammation/fibrosis are critical for the evolution of Systemic Sclerosis (SSc), as well as for the severity of multi-organ involvement that varies widely among patients. Our objective was to evaluate possible changes in the extent of skin involvement and global disease activity in Greek SSc patients treated with bosentan during the CURE study.Material and Methods CURE was a multicenter, prospective, observational clinical study ( NCT05168215) that recruited 211 patients with SSc, having either active or a history of digital ulcers. There were 118 patients with diffuse (56%) and 93 (44%) with limited SSc, 179 (84%) were females and 32 males, and had a mean age of 56 years (±13) and a mean disease duration of 108 months (±85). All patients received bosentan, up to 125 mg twice daily, in addition to various regimens of standard medications for SSc. The study observational period consisted of 4 follow-up visits (scheduled at 4, 8, 12 and 18 months after baseline). Skin involvement was assessed using the Modified Rodnan Skin Score (MRSS). SSc disease activity was evaluated with the EUSTAR activity index score. Univariate analysis was conducted to explore associations between patient characteristics or concomitant therapies and study outcomes.Results Of 211 recruited patients 193 completed the study. At baseline, the mean MRSS was 14.10 (±9.60) and decreased to 13.50 (±9.50) at 18 months, resulting in a mean change of -0.60 (p=0.001). Regarding the EUSTAR activity index, a reduction of 0.8 points was observed (p<0.001); from 3.0 (±2.2) at baseline to 2.2 (±2.0) at 18 months. Univariate analysis showed that patients receiving prostanoids within 6 months prior to baseline as well as those treated with cyclophosphamide during the observation period, had a greater reduction in MRSS at last visit (p=0.001 and p=0.011, respectively). No new safety signals were observed; 2 patients (0.9%) discontinued the study due to adverse events and 2 patients (0.9%) died during the follow-up period.Conclusions The CURE observational study showed an improvement in both MRSS and EUSTAR activity index in Greek patients with SSc treated with bosentan over 18 months.",
  "authors": [
    {
      "affiliations": [
        "First Department of Propaedeutic Internal Medicine, Joint Academic Rheumatology Program, School of Medicine, NKUA, Athens, Greece"
      ],
      "name": "Katerina Laskari"
    },
    {
      "affiliations": [
        "2nd Department of Medicine and Laboratory, Clinical Immunology-Rheumatology Unit, School of Medicine, NKUA, Athens, Greece"
      ],
      "name": "Dimitrios Vassilopoulos"
    },
    {
      "affiliations": [
        "4th Department of Medicine, Aristotle University, Thessaloniki, Greece"
      ],
      "name": "Theodoros Dimitroulas"
    },
    {
      "affiliations": [
        "Department of Rheumatology, Patras University Hospital, University of Patras Medical School, Patras, Greece"
      ],
      "name": "Dimitrios Daoussis"
    },
    {
      "affiliations": [
        "Department of Rheumatology, School of Health Sciences, Faculty of Medicine, University of Ioannina, Ioannina, Greece"
      ],
      "name": "Paraskevi Voulgari"
    },
    {
      "affiliations": [
        "Rheumatology Clinic, Naval Hospital of Athens, Athens, Greece"
      ],
      "name": "Gkikas Katsifis"
    },
    {
      "affiliations": [
        "Rheumatology and Clinical Immunology, University Hospital of Heraklion and University of Crete Medical School, Heraklion, Greece"
      ],
      "name": "George Bertsias"
    },
    {
      "affiliations": [
        "Rheumatology and Clinical Immunology, University Hospital of Heraklion and University of Crete Medical School, Heraklion, Greece"
      ],
      "name": "Argiro Repa"
    },
    {
      "affiliations": [
        "Department of Rheumatology, Evangelismos General Hospital, Athens, Greece"
      ],
      "name": "George Katsikas"
    },
    {
      "affiliations": [
        "First Department of Internal Medicine, Papageorgiou General Hospital, Thessaloniki, Greece"
      ],
      "name": "Nikolaos Papadopoulos"
    },
    {
      "affiliations": [
        "Private Rheumatology Site, Athens, Greece"
      ],
      "name": "Theofanis Karageorgas"
    },
    {
      "affiliations": [
        "Medical Department, ELPEN Pharmaceutical Co. Inc., Athens, Greece"
      ],
      "name": "Polyanthi Papanastasiou"
    },
    {
      "affiliations": [
        "Medical Department, ELPEN Pharmaceutical Co. Inc., Athens, Greece"
      ],
      "name": "Georgios Tsioumas"
    },
    {
      "affiliations": [
        "Department of Rheumatology and Clinical Immunology, Faculty of Medicine, University of Thessaly, Larissa, Greece"
      ],
      "name": "Theodora Simopoulou"
    },
    {
      "affiliations": [
        "Department of Rheumatology and Clinical Immunology, Faculty of Medicine, University of Thessaly, Larissa, Greece"
      ],
      "name": "Dimitrios Bogdanos"
    },
    {
      "affiliations": [
        "First Department of Propaedeutic Internal Medicine, Joint Academic Rheumatology Program, School of Medicine, NKUA, Athens, Greece"
      ],
      "name": "Petros P Sfikakis"
    }
  ],
  "title": "P.280 Evaluation of skin involvement and disease activity in Greek patients with systemic sclerosis treated with bosentan during a 18-month follow-up period: results from the cure clinical study",
  "uid": "21cdab8d-3cb2-5e21-ade3-8481e60d3611"
}