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Undifferentiated connective tissue disease: state of the art on clinical practice guidelines
  1. Margarida Antunes1,
  2. Carlo Alberto Scirè2,
  3. Rosaria Talarico3,
  4. Tobias Alexander4,
  5. Tadej Avcin5,
  6. Chiara Belocchi6,
  7. Andrea Doria7,
  8. Franco Franceschini8,
  9. Ilaria Galetti9,
  10. Marcello Govoni10,
  11. Eric Hachulla11,
  12. David Launay11,
  13. Gemma Lepri12,
  14. Carla Macieira13,
  15. Marco Matucci-Cerinic12,
  16. Carlo Maurizio Montecucco14,
  17. Maria Francisca Moraes-Fontes1,
  18. Luc Mouthon15,
  19. Sabrina Paolino16,
  20. Veronique Ramoni14,
  21. Chiara Tani3,
  22. Sander W Tas17,
  23. Angela Tincani8,
  24. Ronald Van Vollenhoven18,
  25. Margherita Zen7,
  26. Joao Fonseca19,
  27. Stefano Bombardieri19,
  28. João Eurico Fonseca13,
  29. Matthias Schneider20,
  30. Vanessa Smith21,22,
  31. Maurizio Cutolo16,
  32. Marta Mosca3,23 and
  33. Lorenzo Beretta6
  1. 1 Autoimmune Disease Unit (Unidade de Doenças Auto-imunes/Medicina 7.2), Hospital de Curry Cabral, Centro Hospitalar de Lisboa Central, Lisboa, Portugal
  2. 2 Section of Rheumatology, Department of Medical Sciences, University of Ferrara, Ferrara, Italy
  3. 3 Rheumatology Unit, AOU Pisana, Pisa, Italy
  4. 4 Department of Rheumatology and Clinical Immunology, Charité University Hospital Berlin, Berlin, Germany
  5. 5 Department of Allergology, Rheumatology and Clinical Immunology, University Children’s Hospital, University Medical Centre Ljubljana, Ljubljana, Slovenia
  6. 6 Referral Center for Systemic Autoimmue Diseases, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico di Milano, Milan, Italy
  7. 7 Rheumatology Unit, Department of Medicine, AO Padova and University of Padua, Padua, Italy
  8. 8 Rheumatology and Clinical Immunology Unit, Civil Hospital, Brescia, Italy
  9. 9 FESCA, Federation of European Scleroderma Associations, Milan, Italy
  10. 10 Infectious Diseases Unit, Department of Medical Sciences, University of Ferrara and S Anna University Hospital, Ferrara, Italy
  11. 11 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
  12. 12 Department of Clinical and Experimental Medicine, Division of Rheumatology and Scleroderma Unit, AOU Careggi, University of Florence, Florence, Italy
  13. 13 Rheumatology Department, Hospital de Santa Maria, Centro Hospitalar Lisboa Norte, Lisbon Academic Medical Centre, Libson, Portugal
  14. 14 Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foundation, Pavia, Italy
  15. 15 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
  16. 16 Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, IRCCS Polyclinic Hospital San Martino, University of Genoa, Genoa, Italy
  17. 17 Amsterdam Rheumatology & Immunology Center (ARC), Academic Medical Center, Amsterdam, The Netherlands
  18. 18 Clinical Immunology & Rheumatology, Amsterdam Rheumatology & Immunology Center, Academic Medical Center/University of Amsterdam, Amsterdam, The Netherlands
  19. 19 University of Pisa, Pisa, Italy
  20. 20 Department of Rheumatology, Universitätsklinikum Düsseldorf, Düsseldorf, Germany
  21. 21 Department of Rheumatology, Ghent University Hospital, Ghent, Belgium
  22. 22 Department of Internal Medicine, Ghent University, Ghent, Belgium
  23. 23 Rheumatology Unit, University of Pisa, Pisa, Italy
  1. Correspondence to Dr Lorenzo Beretta; lorberimm{at}


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.

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

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  • Contributors All the authors critically reviewed and approved the manuscript and gave a substantial contribution to its drafting.

  • Funding This publication was funded by the European Union’s Health Programme (2014-2020).

  • Disclaimer ERN ReCONNET is one of the 24 European Reference Networks (ERNs) approved by the ERN Board of Member States. The ERNs are co-funded by the European Commission. The content of this publication represents the views of the authors only and it is their sole responsibility; it cannot be considered to reflect the views of the European Commission and/or the Consumers, Health, Agriculture and Food Executive Agency (CHAFEA) or any other body of the European Union. The European Commission and the Agency do not accept any responsibility for use that may be made of the information it contains.

  • Competing interests None declared.

  • Patient consent Not required.

  • Provenance and peer review Commissioned; externally peer reviewed.

  • Data sharing statement No additional data are available.

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