{
  "abstract": "Introduction In patients with a very early diagnosis of systemic sclerosis (VEDOSS), subtle pulmonary changes may precede the development of overt disease. This study aimed to assess whether early functional decline, defined as a 10% reduction in forced vital capacity (FVC), occurs independently of clinical evolution to definite systemic sclerosis (SSc), and to identify baseline predictors of lung function loss.Material and Methods We recruited patients fulfilling VEDOSS criteria, characterized by Raynaud’s phenomenon and SSc-specific features (autoantibodies and/or scleroderma nailfold capillaroscopy), excluding subjects with puffy fingers at baseline. Patients were classified according to evolution to definite SSc during follow-up (evolved vs non-evolved), and occurrence of a significant lung function decline. This stratification generated four subgroups: evolved decliners, evolved non-decliners, non-evolved decliners, and non-evolved non-decliners. We defined a significant functional deterioration as a decline (quantified as percentage change from baseline) of 10% in FVC.Results **68 subjects were enrolled (64 females), with a mean age of 48 ± 14 years. The median baseline FVC was 3.5 L (IQR 3.1–4.1), corresponding to 114% predicted (IQR 100–123). Over a mean follow-up of 3 ± 1.8 years, 21 patients (31%) evolved to definite SSc. At baseline, no significant difference in FVC was found between patients who developed SSc and those who did not (p = 0.69).When stratifying by both evolution and functional decline, baseline FVC was significantly higher in patients who neither declined nor evolved compared with those who both declined and evolved (4.33 vs 3.53 L, p = 0.0023). Among the subgroup of patients who evolved to SSc, baseline FVC was also higher in non-decliners compared with decliners (4.34 vs 3.46 L, p = 0.012). Baseline ESR showed a modest negative correlation with FVC (rho –0.27, p = 0.03).However, when comparing decliners and non-decliners, ESR values did not differ significantly. Kaplan–Meier analyses comparing time to FVC decline between evolved and non-evolved patients (p = 0.39), and time to clinical evolution between decliners and non-decliners (p = 0.86), showed no significant differences.Conclusions In our cohort FVC decline occurred independently of clinical evolution to SSc. Among evolved patients, a subgroup showed lower baseline FVC and greater subsequent loss. Decline occurred despite preserved baseline volumes, showing that preserved FVC did not exclude early functional involvement. ESR correlated weakly with FVC and did not distinguish decliners, confirming the need for longitudinal studies to refine risk stratification in VEDOSS.",
  "authors": [
    {
      "affiliations": [
        "Respiratory Unit and Adult Cystic Fibrosis Center, Fondazione IRCCS Ca Granda Ospedale Maggiore Policlinico, Milan, Italy"
      ],
      "name": "Fiammetta Danzo"
    },
    {
      "affiliations": [
        "Fondazione IRCCS Ca Granda Ospedale Maggiore Policlinico di Milano, Referral Centre for Systemic Autoimmune Diseases, Milan, Italy"
      ],
      "name": "Martina Iacobino"
    },
    {
      "affiliations": [
        "Fondazione IRCCS Ca Granda Ospedale Maggiore Policlinico di Milano, Referral Centre for Systemic Autoimmune Diseases, Milan, Italy"
      ],
      "name": "Lorenzo Beretta"
    },
    {
      "affiliations": [
        "Fondazione IRCCS Ca Granda Ospedale Maggiore Policlinico di Milano, Referral Centre for Systemic Autoimmune Diseases, Milan, Italy"
      ],
      "name": "Marta Mozzo"
    },
    {
      "affiliations": [
        "Respiratory Unit and Adult Cystic Fibrosis Center, Fondazione IRCCS Ca Granda Ospedale Maggiore Policlinico, Milan, Italy"
      ],
      "name": "Antonella Arcadu"
    },
    {
      "affiliations": [
        "Respiratory Unit and Adult Cystic Fibrosis Center, Fondazione IRCCS Ca Granda Ospedale Maggiore Policlinico, Milan, Italy"
      ],
      "name": "Silvia Dellafiore"
    },
    {
      "affiliations": [
        "Respiratory Unit and Adult Cystic Fibrosis Center, Fondazione IRCCS Ca Granda Ospedale Maggiore Policlinico, Milan, Italy"
      ],
      "name": "Marco Mantero"
    },
    {
      "affiliations": [
        "Respiratory Unit and Adult Cystic Fibrosis Center, Fondazione IRCCS Ca Granda Ospedale Maggiore Policlinico, Milan, Italy"
      ],
      "name": "Letizia Morlacchi"
    },
    {
      "affiliations": [
        "Respiratory Unit and Adult Cystic Fibrosis Center, Fondazione IRCCS Ca Granda Ospedale Maggiore Policlinico, Milan, Italy"
      ],
      "name": "Francesco Blasi"
    },
    {
      "affiliations": [
        "Leeds Institute of Rheumatic and Musculoskeletal Medicine, LIRMM, Leeds, United Kingdom",
        "NIHR Leeds Biomedical Research Centre, Leeds Teaching Hospitals Trust, Leeds, United Kingdom"
      ],
      "name": "Francesco Del Galdo"
    },
    {
      "affiliations": [
        "Fondazione IRCCS Ca Granda Ospedale Maggiore Policlinico di Milano, Referral Centre for Systemic Autoimmune Diseases, Milan, Italy",
        "Department of Clinical Sciences and Community Health, Dipartimento di Eccellenza 2023-2027, Milan, Italy"
      ],
      "name": "Chiara Bellocchi"
    }
  ],
  "title": "P.077 Preclinical lung volume loss in very early diagnosis of systemic sclerosis (VEDOSS): characterization of early forced vital capacity (FVC) decline and clinical evolution",
  "uid": "48633700-921a-5757-928a-9f0e91e5f15d"
}