{
  "abstract": "Introduction Systemic sclerosis (SSc) is associated with increased mortality. Intensive therapies have emerged for severe or refractory cases, but carry significant risks. The RESIST score (Risk score for Early mortality to Stratify for Intensive SSc Therapy) was developed to predict early mortality (<5 years) in patients eligible for such treatments.Material and Methods Using the EUSTAR cohort, patients unsuitable for intensive therapy were excluded. Survival was analyzed via Kaplan–Meier estimates. A multivariable Cox model with adaptive LASSO selection was built, informed by expert opinion, univariable analyses, and literature. Model performance was evaluated using the C-index, area under the curve (AUC) at 3 and 5 years, and calibration. Missing data were imputed, and external validation was performed on 6,251 excluded patients.Results Of 22,059 EUSTAR patients, 4,526 met inclusion criteria; 138 died within five years. Deceased patients were more often male (28% vs. 16%), older (53 vs. 49 years), and had diffuse cutaneous SSc (dcSSc) (61% vs. 35%) compared to survivors. The RESIST score included: male sex; dcSSc; age >55 years; elevated CRP; digital ulcers; modified Rodnan skin score >14; left ventricular ejection fraction <60%; and diffusing capacity of the lungs <60%. This allowed SSc patients to be discriminated into three groups with the following five-year survival rates: low risk (99%, 95% CI: 98-100), intermediate risk (96%, 95% CI: 95-97) and high risk (82%, 95% CI: 78-87).The model showed good discrimination in both the development and discrimination cohorts (AUC: 0.79 [0.77–0.79] and 0.78 [0.77–0.79], respectively).Conclusions The RESIST score reliably predicts early mortality in SSc patients eligible for intensive therapies and may guide treatment decisions by identifying those at high risk of death.Abstract P.366 Table 1",
  "authors": [
    {
      "affiliations": [
        "Department of Haematology, Oncology, Clinical Immunology and Rheumatology, University Hospital Tübingen, Tübingen, Germany"
      ],
      "name": "Ann-Christin Pecher"
    },
    {
      "affiliations": [
        "Unit of Clinical Research, Paris Seine Saint Denis University, Bobigny, France"
      ],
      "name": "Marouane Boubaya"
    },
    {
      "affiliations": [
        "Rheumatology Department, Cochin Hospital, Paris, France"
      ],
      "name": "Allanore Yannick"
    },
    {
      "affiliations": [
        "Department of Rheumatology, Ghent University Hospital, Ghent, Belgium"
      ],
      "name": "Vanessa Smith"
    },
    {
      "affiliations": [
        "Department of Rheumatology, Leids Universitair Medisch Centrum, Leiden, The Netherlands"
      ],
      "name": "Jeska de Vries-Bouwstra"
    },
    {
      "affiliations": [
        "Department of Rheumatology, First Faculty of Medicine, Charles University in Prague and Rheumatology Institute, Prague, Czech Republic"
      ],
      "name": "Radim Becvar"
    },
    {
      "affiliations": [
        "Clinica Medica, Department of Internal Medicine, Marche University Hospital, Ancona, Italy"
      ],
      "name": "Gianluca Moroncini"
    },
    {
      "affiliations": [
        "Univ. Lille, Inserm, CHU Lille, U - INFINITE - Institute for Translational Research in Inflammation, Lille, France"
      ],
      "name": "David Launay"
    },
    {
      "affiliations": [
        "Department of Biomedical Sciences, Humanitas University, Pieve Emanuele, Italy",
        "Rheumatology and Clinical Immunology,, Rozzano, Italy"
      ],
      "name": "Maria De Santis"
    },
    {
      "affiliations": [
        "Rheumatology Department, Waikato Hospital, Hamilton, New Zealand"
      ],
      "name": "Kamal Solanki"
    },
    {
      "affiliations": [
        "Department of Internal Medicine and Therapeutics, Università di Pavia, Pavia, Italy"
      ],
      "name": "Carlomaurizio Montecucco"
    },
    {
      "affiliations": [
        "Rheumatology Section, Department of Medicine, University of Verona, Verona, Italy"
      ],
      "name": "Luca Idolazzi"
    },
    {
      "affiliations": [
        "Department of Rheumatology ,Rehabilitation & physical medicine, Assiut University Hospital, Assiut, Egypt"
      ],
      "name": "Nihal Fathi"
    },
    {
      "affiliations": [
        "Department of Internal Medicine, Rheumatology and Clinical Immunology, Medical University of Silesia, Katowice, Poland"
      ],
      "name": "Kotyla Przemyslaw"
    },
    {
      "affiliations": [
        "Department of Rheumatology, UMR ImmunoConcept, Bordeaux University, Bordeaux University Hospital, Bordeaux, France"
      ],
      "name": "Marie-Elise Truchetet"
    },
    {
      "affiliations": [
        "Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan"
      ],
      "name": "Masataka Kuwana"
    },
    {
      "affiliations": [
        "Faculty of Medicine and Health, Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK"
      ],
      "name": "Francesco Del Gado"
    },
    {
      "affiliations": [
        "Department of Rheumatology, University Hospital Zurich, Zürich, Switzerland"
      ],
      "name": "Distler Oliver"
    },
    {
      "affiliations": [
        "Department of Rheumatology, University Hospital Zurich, Zürich, Switzerland"
      ],
      "name": "Muriel Elhai"
    },
    {
      "affiliations": [
        "Department of Haematology, Oncology, Clinical Immunology and Rheumatology, University Hospital Tübingen, Tübingen, Germany"
      ],
      "name": "Jörg Henes"
    }
  ],
  "title": "P.366 Risk score for early mortality to stratify for intensive SSc therapy. The resist score",
  "uid": "c4371086-2bd0-5735-ade7-0c2efb2edd34"
}