{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,28]],"date-time":"2026-10-28T18:10:07Z","timestamp":1793211007690,"version":"4.5.0"},"reference-count":13,"publisher":"BMJ","issue":"Suppl 1","funder":[{"name":"European Union\u2019s Health Programme","award":["Agreement number 739531"],"award-info":[{"award-number":["Agreement number 739531"]}]}],"content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["RMD Open"],"accepted":{"date-parts":[[2019,1,2]]},"published-print":{"date-parts":[[2019,2]]},"abstract":"<jats:p>The term \u2018undifferentiated connective tissue disease\u2019 (UCTD) is generally used to describe clinical entities characterised by clinical and serological manifestations of systemic autoimmune diseases but not fulfilling the criteria for defined connective tissue diseases (CTDs). In this narrative review, we summarise the results of a systematic literature research, which was performed as part of the ERN ReCONNET project, aimed at evaluating existing clinical practice guidelines (CPGs) or recommendations.<\/jats:p>\n                  <jats:p>No specific CPG on UCTD were found, potential areas of intervention are absence of a consensus definition of UCTD, need for specific monitoring and therapeutic protocols, stratification of UCTD based on the risk of developing a defined CTD and preventive measure for the future development of a more severe condition.<\/jats:p>\n                  <jats:p>Patients feel uncertainty regarding the name of the disease and feel the need of a better education and understanding of these conditions and its possible changes over time.<\/jats:p>","DOI":"10.1136\/rmdopen-2018-000786","type":"journal-article","created":{"date-parts":[[2020,5,8]],"date-time":"2020-05-08T03:27:44Z","timestamp":1588908464000},"page":"e000786","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":54,"title":["Undifferentiated connective tissue disease: state of the art on clinical practice guidelines"],"prefix":"10.1136","volume":"4","author":[{"given":"Margarida","family":"Antunes","sequence":"first","affiliation":[{"name":"Autoimmune Disease Unit (Unidade de Doen\u00e7as Auto-imunes\/Medicina 7.2), Hospital de Curry Cabral, Centro Hospitalar de Lisboa Central, Lisboa, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7451-0271","authenticated-orcid":false,"given":"Carlo Alberto","family":"Scir\u00e8","sequence":"additional","affiliation":[{"name":"Section of Rheumatology, Department of Medical Sciences, University of Ferrara, Ferrara, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rosaria","family":"Talarico","sequence":"additional","affiliation":[{"name":"Rheumatology Unit, AOU Pisana, Pisa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1193-0097","authenticated-orcid":false,"given":"Tobias","family":"Alexander","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Clinical Immunology, Charit\u00e9 University Hospital Berlin, Berlin, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tadej","family":"Avcin","sequence":"additional","affiliation":[{"name":"Department of Allergology, Rheumatology and Clinical Immunology, University Children\u2019s Hospital, University Medical Centre Ljubljana, Ljubljana, Slovenia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chiara","family":"Belocchi","sequence":"additional","affiliation":[{"name":"Referral Center for Systemic Autoimmue Diseases, Fondazione IRCCS Ca\u2019 Granda Ospedale Maggiore Policlinico di Milano, Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrea","family":"Doria","sequence":"additional","affiliation":[{"name":"Rheumatology Unit, Department of Medicine, AO Padova and University of Padua, Padua, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Franco","family":"Franceschini","sequence":"additional","affiliation":[{"name":"Rheumatology and Clinical Immunology Unit, Civil Hospital, Brescia, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ilaria","family":"Galetti","sequence":"additional","affiliation":[{"name":"FESCA, Federation of European Scleroderma Associations, Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marcello","family":"Govoni","sequence":"additional","affiliation":[{"name":"Infectious Diseases Unit, Department of Medical Sciences, University of Ferrara and S Anna University Hospital, Ferrara, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eric","family":"Hachulla","sequence":"additional","affiliation":[{"name":"D\u00e9partement de M\u00e9decine Interne et Immunologie Clinique, Centre de R\u00e9f\u00e9rence des Maladies Syst\u00e9miques et Auto-Immunes Rares du Nord-Ouest (CERAINO), LIRIC, INSERM, Univ Lille, CHU Lille, Lille, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David","family":"Launay","sequence":"additional","affiliation":[{"name":"D\u00e9partement de M\u00e9decine Interne et Immunologie Clinique, Centre de R\u00e9f\u00e9rence des Maladies Syst\u00e9miques et Auto-Immunes Rares du Nord-Ouest (CERAINO), LIRIC, INSERM, Univ Lille, CHU Lille, Lille, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gemma","family":"Lepri","sequence":"additional","affiliation":[{"name":"Department of Clinical and Experimental Medicine, Division of Rheumatology and Scleroderma Unit, AOU Careggi, University of Florence, Florence, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carla","family":"Macieira","sequence":"additional","affiliation":[{"name":"Rheumatology Department, Hospital de Santa Maria, Centro Hospitalar Lisboa Norte, Lisbon Academic Medical Centre, Libson, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marco","family":"Matucci-Cerinic","sequence":"additional","affiliation":[{"name":"Department of Clinical and Experimental Medicine, Division of Rheumatology and Scleroderma Unit, AOU Careggi, University of Florence, Florence, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carlo Maurizio","family":"Montecucco","sequence":"additional","affiliation":[{"name":"Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation, Pavia, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8917-6592","authenticated-orcid":false,"given":"Maria Francisca","family":"Moraes-Fontes","sequence":"additional","affiliation":[{"name":"Autoimmune Disease Unit (Unidade de Doen\u00e7as Auto-imunes\/Medicina 7.2), Hospital de Curry Cabral, Centro Hospitalar de Lisboa Central, Lisboa, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Luc","family":"Mouthon","sequence":"additional","affiliation":[{"name":"Service de M\u00e9decine Interne, H\u00f4pital Cochin, Centre de R\u00e9f\u00e9rence Maladies syst\u00e9miques Autoimmunes Rares d\u2019Ile de France, Assistance Publique-H\u00f4pitaux de Paris (AP-HP), Universit\u00e9 Paris Descartes, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sabrina","family":"Paolino","sequence":"additional","affiliation":[{"name":"Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, IRCCS Polyclinic Hospital San Martino, University of Genoa, Genoa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Veronique","family":"Ramoni","sequence":"additional","affiliation":[{"name":"Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation, Pavia, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chiara","family":"Tani","sequence":"additional","affiliation":[{"name":"Rheumatology Unit, AOU Pisana, Pisa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sander W","family":"Tas","sequence":"additional","affiliation":[{"name":"Amsterdam Rheumatology & Immunology Center (ARC), Academic Medical Center, Amsterdam, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Angela","family":"Tincani","sequence":"additional","affiliation":[{"name":"Rheumatology and Clinical Immunology Unit, Civil Hospital, Brescia, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ronald","family":"Van Vollenhoven","sequence":"additional","affiliation":[{"name":"Clinical Immunology & Rheumatology, Amsterdam Rheumatology & Immunology Center, Academic Medical Center\/University of Amsterdam, Amsterdam, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Margherita","family":"Zen","sequence":"additional","affiliation":[{"name":"Rheumatology Unit, Department of Medicine, AO Padova and University of Padua, Padua, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joao","family":"Fonseca","sequence":"additional","affiliation":[{"name":"University of Pisa, Pisa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stefano","family":"Bombardieri","sequence":"additional","affiliation":[{"name":"University of Pisa, Pisa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jo\u00e3o Eurico","family":"Fonseca","sequence":"additional","affiliation":[{"name":"Rheumatology Department, Hospital de Santa Maria, Centro Hospitalar Lisboa Norte, Lisbon Academic Medical Centre, Libson, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matthias","family":"Schneider","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, Universit\u00e4tsklinikum D\u00fcsseldorf, D\u00fcsseldorf, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vanessa","family":"Smith","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, Ghent University Hospital, Ghent, Belgium"},{"name":"Department of Internal Medicine, Ghent University, Ghent, Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maurizio","family":"Cutolo","sequence":"additional","affiliation":[{"name":"Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, IRCCS Polyclinic Hospital San Martino, University of Genoa, Genoa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marta","family":"Mosca","sequence":"additional","affiliation":[{"name":"Rheumatology Unit, AOU Pisana, Pisa, Italy"},{"name":"Rheumatology Unit, University of Pisa, Pisa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6529-6258","authenticated-orcid":false,"given":"Lorenzo","family":"Beretta","sequence":"additional","affiliation":[{"name":"Referral Center for Systemic Autoimmue Diseases, Fondazione IRCCS Ca\u2019 Granda Ospedale Maggiore Policlinico di Milano, Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[2019,2,26]]},"reference":[{"key":"2025090312591524000_4.Suppl_1.e000786.1","doi-asserted-by":"publisher","DOI":"10.1016\/j.autrev.2010.09.013"},{"key":"2025090312591524000_4.Suppl_1.e000786.2","doi-asserted-by":"publisher","DOI":"10.1002\/art.1780230312"},{"key":"2025090312591524000_4.Suppl_1.e000786.3","first-page":"615","article-title":"Undifferentiated connective tissue diseases (UCTD): a review of the literature and a proposal for 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Presentation at: ACR\/ARHP 2017 annual meeting;. European league against rheumatism and American college of rheumatology present new SLE classification criteria at the 2017 ACR\/ARHP annual meeting, San Diego, CA, November 3-8, 2017."},{"key":"2025090312591524000_4.Suppl_1.e000786.10","first-page":"739","article-title":"Undifferentiated connective tissue disease: predictors of evolution into definite disease. in addition, the high prevalence of anti-DFS70 antibodies in the UCTD patients suggested the potential role of these autoantibodies as a marker in the evolution of UCTD to CTD","volume":"35","author":"Garc\u00eda-Gonz\u00e1lez","year":"2017","journal-title":"Clin Exp Rheumatol"},{"key":"2025090312591524000_4.Suppl_1.e000786.11","doi-asserted-by":"publisher","DOI":"10.1002\/acr.22583"},{"key":"2025090312591524000_4.Suppl_1.e000786.12","doi-asserted-by":"crossref","first-page":"591","DOI":"10.1093\/rheumatology\/kex016","article-title":"How to monitor safety and efficacy of biologic treatment in rare, therapy-refractory immune-mediated inflammatory diseases?","volume":"57","author":"Musters","year":"2018","journal-title":"Rheumatology"},{"key":"2025090312591524000_4.Suppl_1.e000786.13","doi-asserted-by":"crossref","DOI":"10.1177\/2333393616664823","article-title":"Trust, health care relationships, and chronic illness: a theoretical coalescence","volume":"3","author":"Robinson","year":"2016","journal-title":"Glob Qual Nurs Res"}],"container-title":["RMD Open"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/rmdopen-2018-000786","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,9,3]],"date-time":"2025-09-03T19:59:23Z","timestamp":1756929563000},"score":1,"resource":{"primary":{"URL":"https:\/\/rmdopen.bmj.com\/lookup\/doi\/10.1136\/rmdopen-2018-000786"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2019,2]]},"references-count":13,"journal-issue":{"issue":"Suppl 1","published-online":{"date-parts":[[2019,2,26]]},"published-print":{"date-parts":[[2019,2]]}},"alternative-id":["10.1136\/rmdopen-2018-000786"],"URL":"https:\/\/doi.org\/10.1136\/rmdopen-2018-000786","relation":{},"ISSN":["2056-5933"],"issn-type":[{"value":"2056-5933","type":"electronic"}],"subject":[],"published":{"date-parts":[[2019,2]]}}}