{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,21]],"date-time":"2026-10-21T19:53:25Z","timestamp":1792612405742,"version":"4.5.0"},"reference-count":46,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2026,3,30]],"date-time":"2026-03-30T00:00:00Z","timestamp":1774828800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"},{"start":{"date-parts":[[2026,3,30]],"date-time":"2026-03-30T00:00:00Z","timestamp":1774828800000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["acrjournals.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["ACR Open Rheumatology"],"published-print":{"date-parts":[[2026,4]]},"abstract":"<jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>\n                      The very early diagnosis of systemic sclerosis (SSc; VEDOSS) criteria aim to identify patients at risk of evolving into SSc. The predictive value of diffusing capacity of the lungs for carbon monoxide (DL\n                      <jats:sc>co)<\/jats:sc>\n                      and forced vital capacity (FVC) remains poorly characterized. This study aimed to evaluate whether baseline pulmonary function test (PFT) alterations, especially DL\n                      <jats:sc>co<\/jats:sc>\n                      , predict progression to SSc and to examine longitudinal PFT changes in patients with stable VEDOSS over time.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>\n                      A retrospective longitudinal study was conducted including 73 patients with VEDOSS. Patients were stratified by serologic, capillaroscopic, and clinical features. Annual PFT follow\u2010up was performed at our center. Progression to SSc was defined according to the 2013 American College of Rheumatology\/EULAR criteria. Kaplan\u2013Meier curves were generated to evaluate progression rates according to baseline DL\n                      <jats:sc>co<\/jats:sc>\n                      \/single breath (SB)%, using 70% as cutoff, and to evaluate time\u2010dependent survival of both DL\n                      <jats:sc>co<\/jats:sc>\n                      % and FVC% decline according to the presence or the absence of SSc\u2010specific antibodies (SSc\u2010Ab), active\u2010late nailfold videocapillaroscopic (NVC) patterns, and puffy fingers. A Cox proportional hazards regression model was used to assess the independent association between baseline DL\n                      <jats:sc>co<\/jats:sc>\n                      \/SB (%) and progression\u2010free survival, adjusting for SSc\u2010Ab+, NVC patterns, and the presence of puffy fingers.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Over a median follow\u2010up of five years (maximum 11 years), 12% of patients transitioned to SSc. All progressors were SSc\u2010Ab+ and had lower baseline DL\n                      <jats:sc>co<\/jats:sc>\n                      \/SB% values (68.2% vs 81.2%,\n                      <jats:italic>P<\/jats:italic>\n                      = 0.004). A DL\n                      <jats:sc>co<\/jats:sc>\n                      \/SB &lt;70% was strongly associated with increased risk of progression toward SSc over time (7.7 vs 10.2 years, log\u2010rank = 0.007). In patients with stable VEDOSS, the time\u2010dependent decline of DL\n                      <jats:sc>co<\/jats:sc>\n                      \/SB% was shorter in SSc\u2010Ab+ group (8 vs 10.4 years, log\u2010rank = 0.032). FVC% also declined in the cohort without any influence of disease\u2010specific variables. Cox regression showed that the SSc\u2010Ab+ and scleroderma NVC pattern were the main contributors to progression toward definite SSc.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>\n                      Baseline DL\n                      <jats:sc>co<\/jats:sc>\n                      \/SB &lt;70% is a potential marker of disease progression in VEDOSS; however, subtle progressive pulmonary function decline may occur even in stable patients.\n                    <\/jats:p>\n                    <jats:p>\n                      <jats:boxed-text content-type=\"graphic\" position=\"anchor\">\n                        <jats:graphic xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" mimetype=\"image\/png\" position=\"anchor\" specific-use=\"enlarged-web-image\" xlink:href=\"graphic\/acr290022-gra-0001-m.png\">\n                          <jats:alt-text>image<\/jats:alt-text>\n                        <\/jats:graphic>\n                      <\/jats:boxed-text>\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/acr2.90022","type":"journal-article","created":{"date-parts":[[2026,3,30]],"date-time":"2026-03-30T14:43:06Z","timestamp":1774881786000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Pulmonary Function Decline in Very Early Diagnosis of Systemic Sclerosis: A Retrospective Longitudinal Single\u2010Center Study"],"prefix":"10.1002","volume":"8","author":[{"ORCID":"https:\/\/orcid.org\/0009-0008-0208-4259","authenticated-orcid":false,"given":"Eugenio","family":"Capparelli","sequence":"first","affiliation":[{"name":"Rheumatology Unit Azienda Socio Sanitaria Territoriale OVEST Milanese, Legnano Hospital  Legnano Milan Italy"},{"name":"Department of Systems Medicine, Rheumatology, Allergology and Clinical Immunology University of Rome Tor Vergata  Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Greta","family":"Pellegrino","sequence":"additional","affiliation":[{"name":"Department of Rheumatology IRCCS Galeazzi\u2010Sant'Ambrogio Hospital  Milan Italy"},{"name":"Department of Biochemical and Clinical Sciences University of Milan  Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eleonora","family":"Zaccara","sequence":"additional","affiliation":[{"name":"Rheumatology Unit Azienda Socio Sanitaria Territoriale OVEST Milanese, Legnano Hospital  Legnano Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giorgio","family":"Bonardi","sequence":"additional","affiliation":[{"name":"Pneumology Unit Azienda Socio Sanitaria Territoriale OVEST Milanese, Legnano Hospital  Legnano Milan 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