{
  "abstract": "Introduction Introduction: Limited cutaneous systemic sclerosis (lcSSc) is the most frequent subset of SSc, affecting 60–80% of all patients, yet it is often underrepresented in clinical trials. One of the challenges remains to identify patients with a high risk of skin progression, in clinical practice and trials. Objectives: To identify predictive parameters of skin progression at 12 ± 3 and 24 ± 3 months in patients with limited and in patients with sine scleroderma.Material and Methods An observational study was conducted using the EUSTAR database. Patients were included if they had lcSSc or sine scleroderma, fulfilled the 2013 ACR/EULAR classification criteria, and had valid data for modified Rodnan skin score (mRSS) at baseline and at 12±3 or 24±3 months follow-up visit.Worsening of skin fibrosis was assessed using three definitions: 1) an increase of 3 points in mRSS; 2) an increase of 5 points in mRSS, 3) or progression to diffuse subset SSc, from baseline to 12 or 24 months follow-up.Univariate logistic regression analyses were performed to identify predictive parameters for skin progression.Results A total of 1,672 and 779 patients with lcSSc or sine SSc were eligible for inclusion at the 12- and 24-month follow-ups, respectively. The median disease duration was 10.5 years, with 28% of patients having a disease duration of less than 3 years and 40% less than 5 years.At 12 months after baseline, 10.2% of patients demonstrated an increase of >3 points, 4.9% an increase of >5 points, and 3.8% progression to the diffuse subset; at 24 months, the corresponding proportions were 11.6%, 6.0%, and 4.0%, respectively.At 12 months, skin worsening (>3 points in mRSS, or progression to diffuse subset) was associated with low DLCO, positive anti-Scl-70, positive anti-RNA polymerase III, negative ACA, baseline mRSS >5, reduced left ejection fraction, tendon friction rubs, low FVC, and higher mDAI scores.At 24 months, predictors included disease duration >7 years, positive anti-Scl-70, higher baseline mRSS, and reduced left ejection fraction.Conclusions Our study provides novel, evidence-based criteria for the enrichment of lcSSc cohorts with patients who experience worsening of skin which allows improved clinical trial design.",
  "authors": [
    {
      "affiliations": [
        "Rheumatology Department, Centro Hospitalar e Universitario de Coimbra, Coimbra, Portugal"
      ],
      "name": "Tania Santiago"
    },
    {
      "affiliations": [
        "Instituto Politécnico de Coimbra, ESTESC-Coimbra Health School, Farmácia, Coimbra, Portugal",
        "QLV Research Consulting, Coimbra, Coimbra, Portugal"
      ],
      "name": "Cristiano Matos"
    },
    {
      "affiliations": [
        "Centre for Musculoskeletal Research, Division of Musculoskeletal and Dermatological Sciences, Manchester, UK",
        "Department of Rheumatology, Northern Care Alliance NHS Foundation Trust, Salford Care Organisation, Salford, UK"
      ],
      "name": "Michael Hughes"
    },
    {
      "affiliations": [
        "Department of Internal Medicine, University of Michigan, Ann Arbor, Michigan, USA"
      ],
      "name": "Dinesh Khanna"
    },
    {
      "affiliations": [
        "Department of Internal Medicine and Clinical Immunology, CHU Rennes, University of Rennes 1, Rennes, France"
      ],
      "name": "Alain Lescoat"
    },
    {
      "affiliations": [
        "EUSTAR Collaborators, EUSTAR, * OTHER"
      ],
      "name": "EUSTAR Collaborators"
    }
  ],
  "title": "P.209 Enrichment criteria for skin progression in future trials: a preliminary analysis of skin progression in limited cutaneous systemic sclerosis and sine scleroderma patients using the EUSTAR cohort",
  "uid": "d621434a-bd36-5e4d-ba38-b698979e3451"
}