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Aim of the study was to revise the UCTD diagnoses and analyse the main predictors of evolution in a monocentric cohort. We retrospectively revised the diagnoses of 331 ANA positive patients, with at least one year of follow-up, classified as UCTD from 2009 and 2017. The diagnosis of UCTD was confirmed in 180 cases (54.4%). The evolution occurred in 18% of cases, after a follow-up of 6.9 (SD: 4.4) years. Raynaud\u2019s phenomenon (RP) (OR: 2.39), puffy hands (OR: 6.3), anti-ENA (OR: 2.34), anti-Topoisomerase I antibodies (OR: 4.93), rheumatoid factor (RF) (OR: 2.86) were associated with evolution. Evolution in Systemic Lupus Erythematosus (SLE) occurred in 5 patients (2.78%) and associated with the addition of new autoantibodies, compared with other evolutions (\n                    <jats:italic>p<\/jats:italic>\n                    : 0.034; OR: 12; 95CI: 1.4\u2013103.4). Evolution in Systemic Sclerosis and pSS was found in 14 (7.8%) and 8 cases (4.4%), respectively. Puffy hands and RF positivity as the predictors of SSc and pSS evolution, respectively. A confirmed diagnosis of UCTD, according with the available criteria, was assessed in about a half patients. The occurrence of puffy hands since the onset defines a patient with a potential evolution into SSc, while the addition of new specific autoantibodies represents a typical \u201cfingerprint\u201d of patients developing SLE.\n                  <\/jats:p>\n                  <jats:p>Trial registration: Studio ANACTD np 1318.<\/jats:p>","DOI":"10.1007\/s10238-025-01614-1","type":"journal-article","created":{"date-parts":[[2025,4,7]],"date-time":"2025-04-07T22:29:08Z","timestamp":1744064948000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Undifferentiated connective tissue disease: the diagnoses critically revised-experience of a single center"],"prefix":"10.1007","volume":"25","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2757-7120","authenticated-orcid":false,"given":"Ilaria","family":"Cavazzana","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7053-3086","authenticated-orcid":false,"given":"Paolo","family":"Semeraro","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3699-8744","authenticated-orcid":false,"given":"Cesare","family":"Tomasi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elena Sofia","family":"Kessler","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0913-0197","authenticated-orcid":false,"given":"Silvia","family":"Piantoni","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8933-7683","authenticated-orcid":false,"given":"Alessia","family":"Caproli","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6511-4936","authenticated-orcid":false,"given":"Micaela","family":"Fredi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3678-6124","authenticated-orcid":false,"given":"Franco","family":"Franceschini","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2025,3,29]]},"reference":[{"key":"1614_CR1","first-page":"1332","volume":"18","author":"GS Alarcon","year":"1991","unstructured":"Alarcon GS, Williams GV, Singer JZ, Steen VD, Clegg DO, Paulus HE, et al. 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Approval was granted by the Local Ethics Committee (Trial registration: Studio ANACTD np 1318).","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethical approval"}},{"value":"Informed consent was obtained from all individual participants included in the study.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent to participate"}}],"article-number":"100"}}