Incidence and predictors of cutaneous manifestations during the early course of systemic sclerosis: a 10-year longitudinal study from the EUSTAR database. Issue 7 (31st July 2015)

• Record Type: Journal Article
• Authors: Wirz, Elina G; Jaeger, Veronika K; Allanore, Yannick; Riemekasten, Gabriela; Hachulla, Eric; Distler, Oliver; Airò, Paolo; Carreira, Patricia E; Tikly, Mohammed; Vettori, Serena; Balbir Gurman, Alexandra; Damjanov, Nemanja; Müller-Ladner, Ulf; Distler, Jörg; Li, Mangtao; Häusermann, Peter; Walker, Ulrich A
• Other Names: author non-byline.; Ananieva Lidia author non-byline.; Heitmann Stefan author non-byline.; Rednic Simona author non-byline.; Jimenez Sergio author non-byline.; Riccieri Valeria author non-byline.; Szmyrka-Kaczmarek Magdalena author non-byline.; Farge Dominique author non-byline.; Lapadula Giovanni author non-byline.; Matucci-Cerinic Marco author non-byline.; Guiducci Serena author non-byline.; Hunzelmann Nicolas author non-byline.; Pozzi Maria Rosa author non-byline.; Mihai Carina author non-byline.; Veale Douglas author non-byline.; Hesselstrand Roger author non-byline.; Mariok Eduardo author non-byline.; Smith Vanessa author non-byline.; Kucharz Eugene J author non-byline.; Czirják László author non-byline.; Martinovic Duska author non-byline.; Solanki Kamal author non-byline.; Ancuta Codrina Mihaela author non-byline.; Sibilia Jean author non-byline.; Paola Caramaschi author non-byline.; Hassanien Manal author non-byline.; Kahl Sarah author non-byline.; Woods Adrianne author non-byline.; Vanthuyne Marie author non-byline.; Ruxandra Ionescu author non-byline.; Radominski Sebastião C author non-byline.; Monaco Andrea Lo author non-byline.; Corrado Ada author non-byline.; Koehm Michaela author non-byline.; Maurizio Montecucco author non-byline.; Radim Bevcar author non-byline.; Loyo Esthela author non-byline.; Üprus Maria author non-byline.; Pellerito Raffaele author non-byline.; Zenone Thierry author non-byline.; Gabrielli Armando author non-byline.; Kowal-Bielecka Otylia author non-byline.; Rozman Blaz author non-byline.; Scorza Raffaella author non-byline.; Saketkoo Lesley Ann author non-byline.; Midtvedt Oyvind author non-byline.; von Mühlen Carlos Alberto author non-byline.; Henes Jörg author non-byline.; Branimir Ani author non-byline.; Hasler Paul author non-byline.; Yavuz Sule author non-byline.; Villiger Peter author non-byline.; Krummel-Lorenz Brigitte author non-byline.; Posa Magdalena author non-byline.; Engelhart Merete author non-byline.; Denton Christopher author non-byline.; Krasowska Dorota author non-byline.; de la Peña Lefebvre Paloma Garcia author non-byline.; Cozzi Franco author non-byline.; Mouthon Luc author non-byline.; Rosato Edoardo author non-byline.; Carlo Selmi author non-byline.; Sancho Juan José Alegre author non-byline.; Mallia Carmel author non-byline.; Limonta Massimiliano author non-byline.; Seidel Matthias author non-byline.; Foti Rosario author non-byline.; Stamp Lisa author non-byline.; Ullman Susanne author non-byline.; Stebbings Simon author non-byline.; Santamaria Vera Ortiz author non-byline.; Del Galdo Francesco author non-byline.; De Langhe Ellen author non-byline.; Mathieu Alessandro author non-byline.; Sunderkötter Cord author non-byline.; Eyerich Kilian author non-byline.; Stamenkovic Bojana author non-byline.; Novak Srdan author non-byline.; Sampaio-Barros Percival D author non-byline.; Kayser Cristiane author non-byline.; Litinsky Ira author non-byline.; Couto Maura author non-byline.;
• Is Part Of: Annals of the rheumatic diseases. Volume 75:Issue 7(2016)
• Page Start: 1285
• Page End: 1292
• Publication Date: 2015-07-31
• Subjects: Systemic Sclerosis -- Epidemiology -- Autoantibodies; Rheumatism -- Periodicals; 616.723005
• DOI: 10.1136/annrheumdis-2015-207271
• Languages: English
• ISSNs: 0003-4967

Abstract

Abstract : Objectives: To longitudinally map the onset and identify risk factors for skin sclerosis and digital ulcers (DUs) in patients with systemic sclerosis (SSc) from an early time point after the onset of Raynaud's phenomenon (RP) in the European Scleroderma Trials and Research (EUSTAR) cohort. Methods: 695 patients with SSc with a baseline visit within 1 year after RP onset were followed in the prospective multinational EUSTAR database. During the 10-year observation period, cumulative probabilities of cutaneous lesions were assessed with the Kaplan–Meier method. Cox proportional hazards regression analysis was used to evaluate risk factors. Results: The median modified Rodnan skin score (mRSS) peaked 1 year after RP onset, and was 15 points. The 1-year probability to develop an mRSS ≥2 in at least one area of the arms and legs was 69% and 25%, respectively. Twenty-five per cent of patients developed diffuse cutaneous involvement in the first year after RP onset. This probability increased to 36% during the subsequent 2 years. Only 6% of patients developed diffuse cutaneous SSc thereafter. The probability to develop DUs increased to a maximum of 70% at the end of the 10-year observation. The main factors associated with diffuse cutaneous SSc were the presence of anti-RNA polymerase III autoantibodies, followed by antitopoisomerase autoantibodies and male sex. The main factor associated with incident DUs was the presence of antitopoisomerase autoantibodies. Conclusion: Early after RP onset, cutaneous manifestations exhibit rapid kinetics in SSc. This should be accounted for in clinical trials aiming to prevent skin worsening.