{
  "abstract": "Introduction Identifying predictors of progression in patients with preclinical systemic sclerosis (PreSSc) and VEDOSS is key to enable early intervention and tailored monitoring. These phases, preceding overt SSc manifestations, remain poorly understood, and robust clinical and biological markers of evolution are lacking. We aimed to define clinical predictors of progression in PreSSc/VEDOSS patients through prospective longitudinal monitoring of a cohort with a well-defined clinical and biological collection. To this end, a close monitoring program with the aim to characterize the PreSSc/VEDOSS risk of progression including biological signatures was set up in 2018 with a planned follow-up of at least 10 years. Here we present the clinical results from a preliminary analysis.Material and Methods We enrolled 62 patients fulfilling PreSSc/VEDOSS criteria, characterized by Raynaud’s phenomenon and SSc-specific features (autoantibodies and/or scleroderma-pattern nailfold capillaroscopy), while excluding subjects with puffy fingers at baseline. Clinical evaluation and instrumental assessments (echocardiography, spirometry, HRCT) were performed every 6 months; biological samples were collected annually. Interval-censored, time-dependent analyses with semi-parametric models were applied to assess predictors of progression to definite SSc according to the ACR/EULAR 2013 criteria.Results Over a mean follow-up of 3 ± 1.8 years, 18 patients (29%) progressed to overt SSc, while 44 (71%) remained preclinical. Among baseline variables included (such as age, duration of Raynaud’s phenomenon, ACA positivity, Scl70 positivity, NVC pattern, and history of reflux), Anti-Scl70 antibodies emerged as the only independent predictor of progression, with a significantly higher risk (HR=3.07, 95% CI=1.41–8.25, p=0.026) ( figure 1). A major clinical hallmark of evolution was the development of interstitial lung disease (ILD), detected by HRCT.Conclusions This prospective study identifies anti-Scl70 positivity as the main baseline determinant of rapid progression in PreSSc/VEDOSS patients. The findings support that all PreSSc/VEDOSS patients should undergo at least annual clinical and instrumental monitoring to capture early disease evolution. Anti-Scl70 positive patients represent a high-risk subgroup and should receive closer surveillance, including HRCT at baseline and upon functional decline, to allow early detection and timely management of ILD. Targeted monitoring strategies may help prevent delayed diagnosis of organ involvement and open new avenues for early therapeutic interventions. Our investigation is still ongoing, and further biological assessments are planned to create a comprehensive picture of SSc preclinical phases.Abstract P.412 Table 1Baseline clinical features in PreSSe/VEDOSS stratified for progressionAbstract P.412 Table 2Clinical features at progression (N=18)Abstract P.412 Figure 1",
  "authors": [
    {
      "affiliations": [
        "Department of Clinical Sciences and Community Health, University of Milan, Dipartimento di Eccellenza 2023-2027, Milan, Italy",
        "Referral Center for Systemic Autoimmune Diseases, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico di Milano, Milan, Italy"
      ],
      "name": "Chiara Bellocchi"
    },
    {
      "affiliations": [
        "Department of Clinical Sciences and Community Health, University of Milan, Dipartimento di Eccellenza 2023-2027, Milan, Italy",
        "Referral Center for Systemic Autoimmune Diseases, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico di Milano, Milan, Italy"
      ],
      "name": "Martina Iacubino"
    },
    {
      "affiliations": [
        "Referral Center for Systemic Autoimmune Diseases, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico di Milano, Milan, Italy"
      ],
      "name": "Marta Mozzo"
    },
    {
      "affiliations": [
        "Department of Clinical Sciences and Community Health, University of Milan, Dipartimento di Eccellenza 2023-2027, Milan, Italy"
      ],
      "name": "Maurizio Lorini"
    },
    {
      "affiliations": [
        "Department of Clinical Sciences and Community Health, University of Milan, Dipartimento di Eccellenza 2023-2027, Milan, Italy"
      ],
      "name": "Vincenzo Carbonelli"
    },
    {
      "affiliations": [
        "Referral Center for Systemic Autoimmune Diseases, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico di Milano, Milan, Italy"
      ],
      "name": "Lorenzo Beretta"
    }
  ],
  "title": "P.412 Longitudinal characterization of PreSSc/VEDOSS patients identifies Anti-Scl70 antibodies as the main predictor of disease progression",
  "uid": "38883a89-3377-5443-9c58-9d345652ac26"
}