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RMD Open. 2019 Feb 26;4(Suppl 1):e000786. doi: 10.1136/rmdopen-2018-000786. eCollection 2018.

Undifferentiated connective tissue disease: state of the art on clinical practice guidelines.

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Autoimmune Disease Unit (Unidade de Doenças Auto-imunes/Medicina 7.2), Hospital de Curry Cabral, Centro Hospitalar de Lisboa Central, Lisboa, Portugal.
Section of Rheumatology, Department of Medical Sciences, University of Ferrara, Ferrara, Italy.
Rheumatology Unit, AOU Pisana, Pisa, Italy.
Department of Rheumatology and Clinical Immunology, Charité University Hospital Berlin, Berlin, Germany.
Department of Allergology, Rheumatology and Clinical Immunology, University Children's Hospital, University Medical Centre Ljubljana, Ljubljana, Slovenia.
Referral Center for Systemic Autoimmue Diseases, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico di Milano, Milan, Italy.
Rheumatology Unit, Department of Medicine, AO Padova and University of Padua, Padua, Italy.
Rheumatology and Clinical Immunology Unit, Civil Hospital, Brescia, Italy.
FESCA, Federation of European Scleroderma Associations, Milan, Italy.
Infectious Diseases Unit, Department of Medical Sciences, University of Ferrara and S Anna University Hospital, Ferrara, Italy.
Département de Médecine Interne et Immunologie Clinique, Centre de Référence des Maladies Systémiques et Auto-Immunes Rares du Nord-Ouest (CERAINO), LIRIC, INSERM, Univ Lille, CHU Lille, Lille, France.
Department of Clinical and Experimental Medicine, Division of Rheumatology and Scleroderma Unit, AOU Careggi, University of Florence, Florence, Italy.
Rheumatology Department, Hospital de Santa Maria, Centro Hospitalar Lisboa Norte, Lisbon Academic Medical Centre, Libson, Portugal.
Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation, Pavia, Italy.
Service de Médecine Interne, Hôpital Cochin, Centre de Référence Maladies systémiques Autoimmunes Rares d'Ile de France, Assistance Publique-Hôpitaux de Paris (AP-HP), Université Paris Descartes, Paris, France.
Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, IRCCS Polyclinic Hospital San Martino, University of Genoa, Genoa, Italy.
Amsterdam Rheumatology & Immunology Center (ARC), Academic Medical Center, Amsterdam, The Netherlands.
Clinical Immunology & Rheumatology, Amsterdam Rheumatology & Immunology Center, Academic Medical Center/University of Amsterdam, Amsterdam, The Netherlands.
University of Pisa, Pisa, Italy.
Department of Rheumatology, Universitätsklinikum Düsseldorf, Düsseldorf, Germany.
Department of Rheumatology, Ghent University Hospital, Ghent, Belgium.
Department of Internal Medicine, Ghent University, Ghent, Belgium.
Rheumatology Unit, University of Pisa, Pisa, Italy.


The term 'undifferentiated connective tissue disease' (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. 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. 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.


clinical practice guidelines; ern reconnet; european reference networks; undifferentiated connective tissue diseases; unmet needs

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