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. Issue 12 (12th August 2013)

• Record Type: Journal Article
• Authors: Minier, Tünde; Guiducci, Serena; Bellando-Randone, Silvia; Bruni, Cosimo; Lepri, Gemma; Czirják, László; Distler, Oliver; Walker, Ulrich A; Fransen, Jaap; Allanore, Yannick; Denton, Christopher; Cutolo, Maurizio; Tyndall, Alan; Müller-Ladner, Ulf; Matucci-Cerinic, Marco
• Other Names: Airò Paolo author non-byline.; Zingarelli Stefania author non-byline.; Ananieva Lidia author non-byline.; Desinova Oxana author non-byline.; Ancuta Codrina Mihaela author non-byline.; Belibou Codruta Iulia author non-byline.; Avouac Jerome author non-byline.; Becvar Radim author non-byline.; Skacelova Simona author non-byline.; Beretta Lorenzo author non-byline.; Vigone Barbara author non-byline.; Caramaschi Paola author non-byline.; Sabbagh Dania author non-byline.; Carpentier Patrick author non-byline.; Damjanov Nemanja author non-byline.; Simic-Pasalic Katarina author non-byline.; Distler Jorg HW author non-byline.; Farge-Bancel Dominique author non-byline.; Hadj-Khelifa Sondess author non-byline.; Foti Rosario author non-byline.; Di Gangi Marcella author non-byline.; de la Pena Lefebvre Paloma Garcia author non-byline.; Hachulla Eric author non-byline.; Salvador Maria Joao author non-byline.; Kayser Cristiane author non-byline.; Camargo Cintia Zumstein author non-byline.; Kumánovics Gábor author non-byline.; Li Mengtao author non-byline.; Xu Dong author non-byline.; Marasini Bianca author non-byline.; Belloli Laura author non-byline.; Maurer Britta author non-byline.; Mayer Miroslav author non-byline.; Mihai Carina author non-byline.; Gherghe Ana Maria author non-byline.; Riccieri Valeria author non-byline.; Stefanantoni Katia author non-byline.; Salsano Felice author non-byline.; Rosato Edoardo author non-byline.; Senecal Jean-Luc author non-byline.; Koenig Martial author non-byline.; Senet Patricia author non-byline.; Frances Camille author non-byline.; Sipek Alenka author non-byline.; Stankovic Aleksandra author non-byline.; Stamenkovic Bojana author non-byline.; Smith Vanessa author non-byline.; Tarner Ingo H author non-byline.; Wiland Piotr author non-byline.;
• Is Part Of: Annals of the rheumatic diseases. Volume 73:Issue 12(2014)
• Page Start: 2087
• Page End: 2093
• Publication Date: 2013-08-12
• Subjects: Systemic Sclerosis -- Outcomes research -- Autoantibodies -- Disease Activity -- Pulmonary Fibrosis; Rheumatism -- Periodicals; 616.723005
• DOI: 10.1136/annrheumdis-2013-203716
• Languages: English
• ISSNs: 0003-4967

Abstract

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.