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
- M Matucci-Cerinic1,
- Y Allanore2,
- L Czirják3,
- A Tyndall4,
- U Müller-Ladner5,
- C Denton6,
- G Valentini7,
- O Distler8,
- K Fligelstone9,
- A Tyrrel-Kennedy9,
- D Farge10,
- O Kowal-Bielecka11,
- F van den Hoogen12,
- M Cutolo13,
- P D Sampaio-Barros14,
- P Nash15,
- K Takehara16,
- D E Furst17
- 1Department of Biomedicine, Division of Rheumatology AOUC, University of Florence, Florence, Italy
- 2Paris Descartes University, Rheumatology A Department, Cochin Hospital, Paris, France
- 3Department of Immunology and Rheumatology, Medical School, University of Pécs, Pécs, Hungary
- 4Department of Rheumatology, University of Basel, Basel, Switzerland
- 5Justus-Liebig University Giessen, Department of Rheumatology and Clinical Immunology, Kerckhoff Clinic Bad Nauheim, Bad Nauheim, Germany
- 6Centre for Rheumatology, Royal Free Hospital, London, UK
- 7Department of Clinical and Experimental Internal Medicine, Rheumatology Unit, Second University of Naples, Naples, Italy
- 8Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland
- 9Federation of the European Scleroderma Associations (FESCA)
- 10Department of Internal Medicine and Vascular Diseases, Assistance Publique-Hôpitaux de Paris, Denis Diderot Paris 7 University, Paris, France
- 11Department of Rheumatology and Internal Medicine, Medical University of Bialystok, Bialystok, Poland
- 12Rheumatology Centre Sint Maartenskliniek, Nijmegen, The Netherlands
- 13Research Laboratories and Clinical Academic Unit of Rheumatology, Department of Internal Medicine, University of Genova, Genova, Italy
- 14Department of Internal Medicine, Unit of Rheumatology, State University of Campinas, Campinas, Brazil
- 15Department of Medicine, University of Queensland, Brisbane, Australia
- 16Department of Dermatology Kanazawa University of Medical Science, Kanazawa, Japan
- 17Geffen School of Medicine, University of California, Los Angeles, USA
- 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}unifi.it
- Accepted 17 April 2009
Abstract
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.
Footnotes
-
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.








