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Preliminary criteria for the very early diagnosis of systemic sclerosis: results of a Delphi Consensus Study from EULAR Scleroderma Trials and Research Group
  1. J Avouac1,
  2. J Fransen2,
  3. UA Walker3,
  4. V Riccieri4,
  5. V Smith5,
  6. C Muller6,
  7. I Miniati7,
  8. IH Tarner8,
  9. S Bellando Randone6,
  10. M Cutolo9,
  11. Y Allanore1,
  12. O Distler10,
  13. G Valentini11,
  14. L Czirjak12,
  15. U Müller-Ladner8,
  16. DE Furst13,
  17. A Tyndall3,
  18. M Matucci-Cerinic7,
  19. EUSTAR Group
  1. 1Rheumatology A Department, Paris Descartes University, Cochin Hospital, Paris, France
  2. 2Department of Rheumatology, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands
  3. 3Department of Rheumatology, Basel University, Basel, Switzerland
  4. 4Sapienza University of Rome, Medical Clinic and Therapy Department, Rome, Italy
  5. 5Department of Rheumatology, Ghent University Hospital, Ghent, Belgium
  6. 6Hospital de Clínicas da Universidade Federal do Paraná, Curitiba, Brazil
  7. 7Department of Medicine, Section of Rheumatology, University of Florence, Florence, Italy
  8. 8Department of Internal Medicine and Rheumatology, Justus-Liebig-University of Giessen, Kerckhoff-Klinik, Bad Nauheim, Germany
  9. 9Department of Internal Medicine, Research Laboratory and Academic Unit of Clinical Rheumatology, University of Genova, Genova, Italy
  10. 10Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland
  11. 11Rheumatology Section, Department of Clinical and Experimental Medicine, Second University of Naples, Naples, Italy
  12. 12Department of Rheumatology, University of Pécs, Pecs, Hungary
  13. 13Division of Rheumatology, Department of Medicine, David Geffen School at UCLA, Los Angeles, California, USA
  1. Correspondence to Dr Jérôme Avouac, Rheumatology A department, Cochin Hospital, 27 rue du Faubourg Saint Jacques, 75014 Paris, France; javouac{at}


Objective To identify a core set of preliminary items considered as important for the very early diagnosis of systemic sclerosis (SSc).

Methods A list of items provided by European League Against Rheumatism (EULAR) Scleroderma Trial and Research(EUSTAR) centres were subjected to a Delphi exercise among 110 experts in the field of SSc. In round 1, experts were asked to choose the items they considered as the most important for the very early diagnosis of SSc. In round 2, experts were asked to reconsider the items accepted after the first stage. In round 3, the clinical relevance of selected items and their importance as measures that would lead to an early referral process were rated using appropriateness scores.

Results Physicians from 85 EUSTAR centres participated in the study and provided an initial list of 121 items. After three Delphi rounds, the steering committee, with input from external experts, collapsed the 121 items into three domains containing seven items, developed as follows: skin domain (puffy fingers/puffy swollen digits turning into sclerodactily); vascular domain (Raynaud's phenomenon, abnormal capillaroscopy with scleroderma pattern) and laboratory domain (antinuclear, anticentromere and antitopoisomerase-I antibodies). Finally, the whole assembly of EUSTAR centres ratified with a majority vote the results in a final face-to-face meeting.

Conclusion The three Delphi rounds allowed us to identify the items considered by experts as necessary for the very early diagnosis of SSc. The validation of these items to establish diagnostic criteria is currently ongoing in a prospective observational cohort.

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  • Competing interests None.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • EUSTAR Group F De Keyser, Department of Rheumatology, Ghent University Hospital, Belgium; A Sulli, C Pizzorni, Research Laboratory and Division of Rheumatology Department of Internal Medicine, University of Genova, Italy; B Maurer, Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland; S Sierakowsky, Department of Rheumatology and Internal Diseases, Medical University of Bialystok, Bialystok, Poland; O Kowal-Bielecka, Department of Rheumatology and Internal Diseases, Medical University of Bialystok, Bialystok, Poland; P Coelho, Instituto portugues de Reumatologia, Lisboa, Portugal; G Riemekasten, Department of Rheumatology-Charité University Hospital, Berlin, Germany; S Rednic, I Nicoara, Clinica Reumatologie – University of Medicine and Pharmacy ‘iuliu Hatieganu’ Cluj, Cluj-Napoca, România; R Caporali, Unità Operativa e Cattedra di Reumatologia, IRCCS Policlinico S, Matteo, Pavia, Italy; J Štork, Department of Dermatology the 1st Faculty of Medicine, Charles University, Prague, Czech Republic; M Inanc, Istanbul Medical Faculty, Department of Internal Medicine, Division of Rheumatology, Istanbul, Turkey; P Carreira, Hospital Universitario 12 de Octubre, Servicio de Reumatología, Madrid, Spain; S Novak, Department of Rheumatology and Clinical Immunology, Internal Medicine, KBC Rijeka, Croatia; C Varju, Department of Immunology and Rheumatology, Faculty of Medicine, University of Pecs, Pecs, Hungary; C Chizzolini, C Ribi, Immunology and Allergy, University Hospital, Geneva, Switzerland; EJ Kucharz, A Kotulska, M Widuchowska, Department of Internal Medicine and Rheumatology, Medical University of Silesia, Katowice, Poland; J Richter, Rheumatology Department, Heinrich-Heine University, Düsseldorf, Germany; A Sipek-Dolnicar, B Rozman, University Medical Center Ljublijana, Division of Internal Medicine, Department of Rheumatology, Ljubljana, Slovenia; A Gabrielli, G Moroncini, Istituto di Clinica Medica Generale, Ematologia ed Immunologia Clinica Polo Didattico Università Politecnica delle Marche, Ancona, Italy; D Farge, C Durant, Department of Internal Medicine, Hopital Saint Louis, Paris, France; HP Kiener, E Rath, Department of Medicine III, Division of Rheumatology, Medical University of Vienna, Vienna, Austria; P Airo, Servizio di Reumatologia e Immunologia Clinica, Spedali Civili, Brescia, Italy; FA Wollheim, Department of Rheumatology, Lund University Hospital, Lund, Sweden; N Hunzelmann, Universitätshautklinik Köln, Köln, Germany; S Bombardieri, A Della Rossa, L Bazzichi, Department of Internal Medicine, Rheumatology Unit, University of Pisa-Santa Chiara, Pisa, Italy; R Pellerito, M Saracco, Ospedale Mauriziano, Centro di Reumatologia, Torino, Italy; C Denton, Centre for Rheumatology, Royal Free and University College London Medical School, London, UK; M Vonk, F Van den Hoogen, Sint Maartenskliniek, Nijmegen, The Netherlands; N Damjanov, Institute of Rheumatology, Belgrade, Serbia and Montenegro; I Kötter, Universitätsklinikum Tübingen, Medizinische Klinik und Poliklinik, Tübingen, Germany; S Heitmann, Department of Rheumatology, Marienhospital Stuttgart, Stuttgart, Germany; M Seidel, Medizinische Univesitäts-Poliklinik, Department of Rheumatology, Bonn, Germany; P Hasler, Kantonsspital Aarau, Rheumaklinik und Institut für Physikalische Medizin und Rehabilitation, Aarau, Switzerland; JM Van Laar, James Cook University Hospital, Middlesbrough, UK; MJ Salvador, JA Pereira Da Silva, Reumatologia, Hospitais da Universidade, Coimbra, Portugal; S Jacobsen, Department of Rheumatology Rigshospitalet, University of Copenhagen, Copenhagen, Denmark; M Worm, Charité – Universitätsmedizin Berlin, Department of Dermatology and Allergy, Berlin, Germany; A Kuhn, Department of Dermatology, University of Münster, Münster, Germany; T Nevskaya, EL Nasonov, Institute of Rheumatology, Russian Academy of Medical Science, Moscow, Russia; R Scorza, UO Immunologia Clinica Centro di Riferimento per le Malattie Autoimmuni Sistemiche, Milano, Italy; H Nielsen, Department of Rheumatology and Endocrinology, Herlev, Denmark; RM Silver, Division of Rheumatology and Immunology, Charleston, South Carolina, USA; E Hachulla, D Launay, Department of Internal Medicine, Hôpital Claude Huriez, Lille, France; G Valesini, Divisione Di Reumatologia – Universita Di Roma La Sapienza, Roma, Italy; R Ionescu, D Opris, ‘Carol Davila’ University of Medicine and Pharmacy, Department of Internal Medicine and Rheumatology, Bucharest, Romania; N Del Papa, W Maglione, D Comina, Rheumatology, Gaetano Pini, Milano, Italy; G Udrea, C Ciurtin, R Ionitescu, C Mihai, Clinic of Internal Medicine and Rheumatology, Dr I Cantacuzino Hospital and Early Arthritis Center, Bucharest, Romania; C Sunderkötter, Department of Dermatology, University of Münster, Münster, Germany; Jae-Bum Jun, Hanyang University Hospital for Rheumatic Diseases, Seoul, Republic of Korea; C Derk, Thomas Jefferson University, Philadelphia, USA; S Alhasani, L Alhajjar, Rheumatology and Rehabilitation Department, Ibnsina Teaching Hospital, Mosul, Iraq; E Ton, University Medical Centre Utrecht, Utrecht, The Netherlands; J Seibold, Division of Rheumatology University of Connecticut Health Center, Farmington, Connecticut, USA; P Nash, Rheumatology Research Unit, Queensland, Australia; L Mouthon, Department of Internal Medicine, Hôpital Cochin, Paris, France; CA von Mühlen, Rheuma Clinic, Porto Alegre, Brazil; B Krummel-Lorenz, P Eilbacher Endokrinologikum, Frankfurt, Germany; R Westhovens, E De Langhe Catholic University of Leuven, Leuven, Belgium; M Mayer, B Anic, M Baresic, University Hospital Centre Zagreb, Division of Clinical Immunology and Rheumatology, Zagreb, Croatia; F Stoeckl, Klinikum Darmstadt, Med Klinik III, Nephrology, Hypertension and Rheumatology, Darmstadt, Germany; M Üprus, East-Tallinn Central Hospital, Department of Rheumatology, Tallinn, Estonia; S Popa, Department of Rheumatology, Republican Clinical Hospital, Chisinau, Republic of Moldova; M Buslau, European Centre for the Rehabilitation of Scleroderma, Rheinfelden, Switzerland; B Granel, Service de Médecine Interne, Hôpital Nord, Marseille, France; T Zenone, Department of Medicine, Unit of Internal Medicine, Valence, France; A Mathieu, A Vacca, II Chair of Rheumatology, University of Cagliari, Monserrato, Italy; P Amerio, Clinica Dermatologica Università di Chieti, Chieti, Italy; T Tourinho, L Lonzetti, M Lemos Lopes, Complexo Hospitalar Santa Casa de Porto Alegre, Porto Alegre, Brazil; R de Souza, University of São Paulo – Rheumatology Division, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil; D Vealex, Department of Rheumatology, Bone and Joint Unit, St Vincent's University Hospital, Dublin, Ireland; P Caramaschi, Dipartimento di Medicina Clinica e Sperimentale, Università di Verona, Verona, Italy; A Balbir-Gurman, Y Braun, Shine Department of Rheumatology, Rambam Health Care Campus, Haifa, Israel; S Ullman, Department of Dermatology, University Hospital of Copenhagen, Copenhagen, Denmark; M Szmyrka-Kaczmarek, E Morgiel, Department of Rheumatology and Internal Diseases, Wroclaw University of Medicine, Wroclaw, Poland; M Vanthuyne, Université catholique de Louvain, Bruxelles, Belgium; M Meurer, P Rehberger, Department of Dermatology, University Medical Center Carl Gustav Carus, Dresden, Germany; C Müller, Universidade Federal do Paraná, Curitiba-Paraná, Brazil; P Sampaio-Barros, University of São Paulo – Rheumatology Division São Paulo, Brazil.