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.
OBJECTIVE
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.
Department of Rheumatology and Immunology, University of Pécs, Pécs, Hungary.
Tünde
T
Minier
Department of Biomedicine, Division of Rheumatology AOUC and Department of Clinical and Experimental Medicine, University of Florence, Florence, Italy.
Serena
S
Guiducci
Department of Biomedicine, Division of Rheumatology AOUC and Department of Clinical and Experimental Medicine, University of Florence, Florence, Italy.
Silvia
S
Bellando-Randone
Department of Biomedicine, Division of Rheumatology AOUC and Department of Clinical and Experimental Medicine, University of Florence, Florence, Italy.
Cosimo
C
Bruni
Department of Biomedicine, Division of Rheumatology AOUC and Department of Clinical and Experimental Medicine, University of Florence, Florence, Italy.
Gemma
G
Lepri
Department of Rheumatology and Immunology, University of Pécs, Pécs, Hungary.
László
L
Czirják
Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland.
Oliver
O
Distler
Department of Rheumatology, University Hospital Basel, Basel, Switzerland.
Ulrich A
UA
Walker
Department of Rheumatology, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands.
Jaap
J
Fransen
Department of Rheumatology, Université Paris Descartes, Paris, France.
Yannick
Y
Allanore
Centre for Rheumatology and Connective Tissue Diseases, UCL Medical School, Royal Free Campus, London, UK.
Christopher
C
Denton
Department of Internal Medicine, University of Genova, Research Laboratory and Academic Clinical Unit of Rheumatology, Viale Benedetto, Italy.
Maurizio
M
Cutolo
Department of Rheumatology, University Hospital Basel, Basel, Switzerland.
Alan
A
Tyndall
Department of Internal Medicine and Rheumatology, Justus-Liebig-University Giessen, Giessen, Germany.
Ulf
U
Müller-Ladner
Department of Biomedicine, Division of Rheumatology AOUC and Department of Clinical and Experimental Medicine, University of Florence, Florence, Italy.
Marco
M
Matucci-Cerinic
D000328:Adult; D000974:Antibodies, Antinuclear / Q000276:immunology; D015331:Cohort Studies; D042241:Early Diagnosis; D005260:Female; D005385:Fingers / Q000098:blood supply / Q000473:pathology*; D006801:Humans; D008297:Male; D020678:Microscopic Angioscopy; D008875:Middle Aged; D011928:Raynaud Disease / Q000150:complications / Q000175:diagnosis* / Q000276:immunology; D012595:Scleroderma, Systemic / Q000150:complications / Q000175:diagnosis* / Q000276:immunology; D012883:Skin Ulcer / Q000150:complications / Q000175:diagnosis* / Q000276:immunology; D013684:Telangiectasis / Q000150:complications / Q000175:diagnosis* / Q000276:immunology
D000974:Antibodies, Antinuclear
Autoantibodies; Disease Activity; Outcomes research; Pulmonary Fibrosis; Systemic Sclerosis