{
  "abstract": "Introduction The Very Early Diagnosis of Systemic Sclerosis (VEDOSS) criteria aim to identify patients at risk of evolving into Systemic Sclerosis. The predictive value of DLCO and FVC remains poorly characterized. This study aimed to evaluate whether baseline pulmonary function test (PFT) alterations, especially DLCO, predict progression to SSc and to examine longitudinal PFT changes in stable VEDOSS patients over time.Material and Methods A retrospective longitudinal study was conducted including 73 VEDOSS patients. Patients were stratified by serological, capillaroscopic, and clinical features. Annual PFT follow-up was performed at our center. Progression to SSc was defined according to the 2013 ACR/EULAR criteria. Kaplan-Meier curves were generated to evaluate progression rates according to baseline DLCO-SB%, using 70% as cut-off, and to evaluate time-dependent survival of both DLCO% and FVC% decline according to the presence or absence of SSc-specific antibodies (SScAb+), active-late versu early/NS capillaroscopic patterns and the presence of puffy fingers. A p less than 0.05 was considered statistically significant.Results Over a median follow-up of 5 years (maximum 11), 12% of patients transitioned to SSc. All progressors were SScAb+ and had lower baseline DLCO/SB% values (68.2% vs 81.2%, p=0.004). A DLCO/SB<70% was strongly associated with increased risk of progression toward SSc over time (7.7 vs 10.2 years, log-rank=0.007). In stable VEDOSS patients, the time-dependent decline of DLCO/SB was shorter in SScAb+ group (8 vs 10.4 years, log-rank=0.032). FVC% also declined in the cohort without any influence of disease specific variables. No significant differences were found in PFT decline rate according NVC patterns and the presence of puffy hands.Conclusions Baseline DLCO/SB<70% is a potential marker of disease progression in VEDOSS, however subtle progressive pulmonary function decline may occur even in stable patients.Abstract P.355 Figure 1Kaplan-Meier curves showing progression-free survival to definite SSe according to baseline DLCO/5B% valuesAbstract P.355 Figure 2Kaplan-Mier survisal crves illustrate the probabiity of free survival firem lang function dicliae ia patiats with stable VEDOSSAbstract P.355 Table 1Patients’ main charncteristics and univariable comparisons between stable VEDOSS and transitioned to SSe patientoAbstract P.355 Table 2Baseline PFT comparisons between stable VEDOSS and transitioned to SSe patients",
  "authors": [
    {
      "affiliations": [
        "Rheumatology Unit, ASST Ovest Milanese, Legnano Hospital, Milan, Italy"
      ],
      "name": "Eugenio Capparelli"
    },
    {
      "affiliations": [
        "Department of Rheumatology, IRCCS Galeazzi-Sant’Ambrogio Hospital, Milan, Italy., Milan, Italy"
      ],
      "name": "Greta Pellegrino"
    },
    {
      "affiliations": [
        "Rheumatology Unit, ASST Ovest Milanese, Legnano Hospital, Milan, Italy"
      ],
      "name": "Eleonora Zaccara"
    },
    {
      "affiliations": [
        "Oneumology Unit, ASST Ovest Milanese, Legnano Hospital, Milan, Italy"
      ],
      "name": "Elisabetta Ricchiuti"
    },
    {
      "affiliations": [
        "Oneumology Unit, ASST Ovest Milanese, Legnano Hospital, Milan, Italy"
      ],
      "name": "Giorgio Bonardi"
    },
    {
      "affiliations": [
        "Oneumology Unit, ASST Ovest Milanese, Legnano Hospital, Milan, Italy"
      ],
      "name": "Daniele Colombo"
    },
    {
      "affiliations": [
        "Rheumatology, Allergology And Clinical Immunology, Department of Medicine of Systems, University of Rome Tor Vergata, Rome"
      ],
      "name": "Maria Sole Chimenti"
    },
    {
      "affiliations": [
        "Rheumatology Unit, ASST Ovest Milanese, Legnano Hospital, Milan, Italy"
      ],
      "name": "Paola Faggioli"
    },
    {
      "affiliations": [
        "Internal Medicine Departement, Asst Ovest Milanese, Legnano Hospital, Milan"
      ],
      "name": "Antonino Mazzone"
    }
  ],
  "title": "P.355 Pulmonary function decline in very early diagnosis of systemic sclerosis (VEDOSS): a retrospective longitudinal single-center study",
  "uid": "321a5944-d4bc-51a0-8123-607513179f13"
}