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Preliminary analysis of the Very Early Diagnosis of Systemic Sclerosis (VEDOSS) EUSTAR multicentre study: evidence for puffy fingers as a pivotal sign for suspicion of systemic sclerosis
  1. Tünde Minier1,
  2. Serena Guiducci2,
  3. Silvia Bellando-Randone2,
  4. Cosimo Bruni2,
  5. Gemma Lepri2,
  6. László Czirják1,
  7. Oliver Distler3,
  8. Ulrich A Walker4,
  9. Jaap Fransen5,
  10. Yannick Allanore6,
  11. Christopher Denton7,
  12. Maurizio Cutolo8,
  13. Alan Tyndall4,
  14. Ulf Müller-Ladner9,
  15. Marco Matucci-Cerinic2,
  16. and the EUSTAR co-workers
  1. 1Department of Rheumatology and Immunology, University of Pécs, Pécs, Hungary
  2. 2Department of Biomedicine, Division of Rheumatology AOUC and Department of Clinical and Experimental Medicine, University of Florence, Florence, Italy
  3. 3Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland
  4. 4Department of Rheumatology, University Hospital Basel, Basel, Switzerland
  5. 5Department of Rheumatology, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands
  6. 6Department of Rheumatology, Université Paris Descartes, Paris, France
  7. 7Centre for Rheumatology and Connective Tissue Diseases, UCL Medical School, Royal Free Campus, London, UK
  8. 8Department of Internal Medicine, University of Genova, Research Laboratory and Academic Clinical Unit of Rheumatology, Viale Benedetto, Italy
  9. 9Department of Internal Medicine and Rheumatology, Justus-Liebig-University Giessen, Giessen, Germany
  1. Correspondence to Professor Marco Matucci-Cerinic, Division of Rheumatology AOUC, Denothe Centre, Department of Biomedicine, University of Florence, Villa Monna Tessa, Viale Pieraccini 18, Florence 50139, Italy; cerinic{at}unifi.it

Abstract

Objectives The EULAR (European League Against Rheumatism) Scleroderma Trials and Research Group (EUSTAR) has identified preliminary criteria for very early diagnosis of systemic sclerosis (SSc). Our aim was to assess the prevalence of each proposed diagnostic item in a large observational patient cohort with Raynaud's phenomenon (RP).

Methods Baseline data of 469 RP patients enrolled into the Very Early Diagnosis of Systemic Sclerosis (VEDOSS) cohort are presented.

Results 68% of all RP patients were antinuclear antibody (ANA) positive. ANA+ RP patients more frequently had previous or current puffy fingers (PuFi) (38.5% and 23.3%, p<0.01) and an SSc pattern on nailfold capillaroscopy (NC) (53.6% and 13.4%, p<0.001) than ANA− patients. Telangiectasia, current digital ulcers and digital pitting scars were also commoner in ANA+ RP patients. 38% of ANA+ patients presented with all three features, which should raise suspicion of very early SSc (ANA+RP+PuFi constitutes a ‘red flag’). These patients more frequently exhibited an NC SSc pattern, sclerodactyly and telangiectases compared to ANA+ patients without PuFi. Almost 90% of patients with ‘red flags’ had anti-centromere or anti-topoisomerase I antibodies and/or an NC SSc pattern, and fulfilled the EUSTAR criteria for very early SSc. Previous or current PuFi were present in 23.3% of ANA− RP patients, eight of whom also had an NC SSc pattern.

Conclusions In addition to well-characterised predictive factors, PuFi is an important sign raising suspicion for underlying very early SSc in patients with RP. The relevance of PuFi in ANA− RP patients should be clarified.

  • Systemic Sclerosis
  • Outcomes research
  • Autoantibodies
  • Disease Activity
  • Pulmonary Fibrosis

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