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The challenge of early systemic sclerosis for the EULAR Scleroderma Trial and Research group (EUSTAR) community. It is time to cut the Gordian knot and develop a prevention or rescue strategy
  1. M Matucci-Cerinic1,
  2. Y Allanore2,
  3. L Czirják3,
  4. A Tyndall4,
  5. U Müller-Ladner5,
  6. C Denton6,
  7. G Valentini7,
  8. O Distler8,
  9. K Fligelstone9,
  10. A Tyrrel-Kennedy9,
  11. D Farge10,
  12. O Kowal-Bielecka11,
  13. F van den Hoogen12,
  14. M Cutolo13,
  15. P D Sampaio-Barros14,
  16. P Nash15,
  17. K Takehara16,
  18. D E Furst17
  1. 1
    Department of Biomedicine, Division of Rheumatology AOUC, University of Florence, Florence, Italy
  2. 2
    Paris Descartes University, Rheumatology A Department, Cochin Hospital, Paris, France
  3. 3
    Department of Immunology and Rheumatology, Medical School, University of Pécs, Pécs, Hungary
  4. 4
    Department of Rheumatology, University of Basel, Basel, Switzerland
  5. 5
    Justus-Liebig University Giessen, Department of Rheumatology and Clinical Immunology, Kerckhoff Clinic Bad Nauheim, Bad Nauheim, Germany
  6. 6
    Centre for Rheumatology, Royal Free Hospital, London, UK
  7. 7
    Department of Clinical and Experimental Internal Medicine, Rheumatology Unit, Second University of Naples, Naples, Italy
  8. 8
    Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland
  9. 9
    Federation of the European Scleroderma Associations (FESCA)
  10. 10
    Department of Internal Medicine and Vascular Diseases, Assistance Publique-Hôpitaux de Paris, Denis Diderot Paris 7 University, Paris, France
  11. 11
    Department of Rheumatology and Internal Medicine, Medical University of Bialystok, Bialystok, Poland
  12. 12
    Rheumatology Centre Sint Maartenskliniek, Nijmegen, The Netherlands
  13. 13
    Research Laboratories and Clinical Academic Unit of Rheumatology, Department of Internal Medicine, University of Genova, Genova, Italy
  14. 14
    Department of Internal Medicine, Unit of Rheumatology, State University of Campinas, Campinas, Brazil
  15. 15
    Department of Medicine, University of Queensland, Brisbane, Australia
  16. 16
    Department of Dermatology Kanazawa University of Medical Science, Kanazawa, Japan
  17. 17
    Geffen School of Medicine, University of California, Los Angeles, USA
  1. Correspondence to Professor M Matucci-Cerinic, Department of Biomedicine, Division of Rheumatology AOUC, Villa Monna Tessa, Viale Pieraccini 18, I-50139 Florence, Italy; cerinic{at}


Early diagnosis of systemic sclerosis (SSc) may allow the start of treatment that could slow disease progression. For this reason early diagnosis of the disease is of pivotal importance. However, the lack of diagnostic criteria and valid predictors significantly limit patient evaluation and the use of potentially effective drugs in the earliest phase of SSc. Early SSc may be suspected on the basis of Raynaud's phenomenon, puffy fingers, autoantibodies and SSc capillaroscopic pattern. In practice, the aim is to have criteria for the diagnosis of very early SSc. The criteria that are proposed are obviously provisional and need to be validated: (a) initially through a Delphi technique; (b) thereafter perhaps using already available datasets; but (c) of critical importance, through prospective studies. Only after prospective studies can these potential criteria be considered validated. The consensus on criteria for the classification of very early SSc might be part of the evolving EULAR/ACR project of reclassification of SSc.

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  • For numbered affiliations see end of article

  • Funding EUSTAR is funded by a grant from EULAR and by an unrestricted grant from Actelion, Encysive and Pfizer.

  • Competing interests None.