{
  "abstract": "Introduction Systemic sclerosis (SSc) is a heterogeneous autoimmune disease characterized by vasculopathy, fibrosis, and autoimmunity. Early diagnosis and intervention in SSc might be of value for improving patient outcomes and preventing severe complications. The introduction of the VEDOSS criteria marked an important step in more accurately identifying convertors in this early, pre-SSc stage. However, they may capture both patients with early disease at risk of progression, and those with longstanding mild disease. The objective of this study was to evaluate the progression from patients meeting the adjusted VEDOSS criteria (all patients had puffy fingers for less than three years), to progressive SSc with skin and/or organ involvement. Specifically, we aimed to assess the incidence of progression and explored several clinical characteristics and biomarkers that predict disease progression. Finally we set out to describe the course in microvascular changes, analyzed through nailfold videocapillaroscopy (NVC).Material and Methods This was a prospective, longitudinal cohort study extending the Hit Hard and Early (HHE) trial, with a 3-year follow-up. Patients were eligible if they had Raynaud’s phenomenon, puffy fingers <3 years, SSc-specific autoantibodies, and an early or active scleroderma pattern on NVC, but no prior organ or skin involvement. The primary endpoint was time to significant disease progression, defined by the presence of any organ or skin involvement, including vascular and musculoskeletal involvement. Kaplan–Meier analyses and univariate Cox regression were used to assess time to progression and identify baseline predictors.Results Of the 30 patients included, 43% (95% CI: 25.6–61.2%) developed clinically relevant disease progression within 3 years, predominantly presenting as digital ulcers and skin thickening. When including milder signs like mRSS > 0-2 and pitting scars, the progression rate increased to 73%. Anti-RNP antibodies were significantly associated with progression (HR 32.5, 95% CI 3.3–314), whereas other clinical and NVC features showed no significant predictive value. Mean capillary density declined over time, and 55% of patients with an early SSc capillaroscopy pattern progressed to an active or late pattern within 24 months. Transition from an active/late pattern to a normal/early pattern was rare (5%, 95% CI 0.1% to 23.8%).Conclusions The high rate of disease progression, especially in a population carefully selected to reflect true early disease, underscores the potential importance of timely recognition and monitoring to enable early intervention. Our findings may help guide the design of future interventional trials aiming at disease interception in SSc.",
  "authors": [
    {
      "affiliations": [
        "Radboudumc, Nijmegen, The Netherlands"
      ],
      "name": "Dieneke Haverkort"
    },
    {
      "affiliations": [
        "Radboudumc, Nijmegen, The Netherlands"
      ],
      "name": "Madelon Vonk"
    }
  ],
  "title": "OC.12 Early progression of systemic sclerosis: a longitudinal cohort study on disease evolution and microvascular changes",
  "uid": "df965c3e-5ba5-5fd9-abda-d54211872724"
}