@article{3003117, title = "Clinical risk assessment of organ manifestations in systemic sclerosis: A report from the EULAR Scleroderma Trials and Research group database", author = "Walker, U.A. and Tyndall, A. and Czirják, L. and Denton, C. and Farge-Bancel, D. and Kowal-Bielecka, O. and Müller-Ladner, U. and Bocelli-Tyndall, C. and Matucci-Cerinic, M. and Riemekasten, G. and Brückner, C. and Airó, P. and Scarsi, M. and Scorza, R. and Beretta, L. and Cozzi, F. and Tiso, F. and Vonk, M.C. and Van Den Hoogen, F.H.J. and Wigley, F.M. and Hummers, L. and Nevskaya, T. and Ananieva, L. and Miniati, I. and Tartaglia, N. and Lomater, C. and Balbir-Gurman, A. and Braun-Moscovici, Y. and Bambara, L.M. and Caramaschi, P. and Valentini, G. and Ruocco, L. and Krieg, T. and Hunzelmann, N. and Varjú, C. and Carriera, P.E. and Joven, B. and Iannone, F. and Lapadula, G. and Kahan, A. and Allanore, Y. and Gabrielli, A. and Imperatore, M. and Scheja, A. and Wollheim, F. and Damjanov, N. and Ostojic, P. and Saar, P. and Tarner, I.H. and Kötter, I. and Bombardieri, S. and Bazzichi, L. and Del Papa, N. and Comina, D.P. and Lo Monaco, A. and La Corte, R. and Hachulla, E. and Launay, D. and Distler, O. and Ciurea, A. and Sierakowski, S. and Mitchell, H. and Silver, R.M. and Krasowska, D. and Michalska-Jakubus, M. and Tikly, M. and Aboo, N. and Worm, M. and Klaus, P. and Rovenský, J. and Lukáčová, O. and Rozman, B. and Sipek, A. and Clemente-Coelho, P. and Shoenfeld, Y. and Langewitch, P. and Da Silva José, A.P. and Salvador, M.J. and Kuhn, A. and Erdmann, G. and Bečvář, R. and Friedl, E. and Graninger, W. and Riccieri, V. and Caporali, R. and Montecucco, C. and Vlachoyiannopoulos, P. and Distler, M. and Reich, K. and Majdan, M. and Wielosz, E. and Rednic, S. and Van Laar, J.M. and Heitmann, S. and Bruckner, A. and Himsel, A. and Riemann, J. and Meyringer, R. and Müller, A. and Martinovic, D. and Radic, M. and Sticherling, M. and Szekanecz, Z. and Szücs, G. and Giacomelli, R. and Marrelli, A. and Stamenkovic, B. and Stankovic, A. and Aringer, M. and Smolen, J.S. and Kucharz, E.J. and Kotulska, A.T. and Jablonska, S. and Blasczik, M. and Jun, J.-B. and Mallia, C. and Coleiro, B. and Santamaria, V.O. and Hinrichs, R. and Nielsen, H. and Cossutta, R. and Ionescu, R. and Opris, D. and Steinbrink, K. and Grundt, B. and Bajocchi, G. and Jiří, Š. and Lefebvre, P.G.D.L.P. and Zea Mendoza, A.C. and Ribi, C. and Chizzolini, C. and Wisłowska, M. and Novak, S. and Indiveri, F. and Jacobsen, S. and Frandsen, P.B. and Gorska, I.Z. and Tore Gran, J. and Midtvedt, Ø. and Ramos, F.O. and Rajcevska, L.D. and Bozinovski, G. and Schöffel, D. and Sunderkötter, C. and Böhm, M. and Morović-Vergles, J. and Čulo, M.-I. and Cutolo, M. and Sulli, A. and Derk, C.T. and Jimenez, S.A. and Siakka, P. and Søndergaard, K. and Stengaard-Pedersen, K. and Cabane, J. and Tiev, K.P. and Mihai, C. and Sfrent-Cornateanu, R. and Jendro, M. and Tuvik, P. and Antivalle, M. and Randisi, G. and Seidel, M. and Clarenbach, R. and Simsek, I. and Dinc, A. and Inanc, M. and Capraru, M.S. and Capraru, D. and Bañegil, I. and Richter, J. and Alhasani, S. and Földvari, I. and Pinto, S. and Brandão, F. and Mas, A.J.", journal = "Annals of the Rheumatic Diseases", year = "2007", volume = "66", number = "6", pages = "754-763", publisher = "BMJ Publishing Group", issn = "0003-4967, 1468-2060", doi = "10.1136/ard.2006.062901", keywords = "centromere antibody, adult; article; clinical assessment; clinical feature; controlled study; data analysis; data base; disease association; disease classification; female; human; information processing; major clinical study; male; organization; prevalence; priority journal; Raynaud phenomenon; risk assessment; scleroderma; systemic sclerosis", abstract = "Background: Systemic sclerosis (SSc) is a multisystem autoimmune disease, which is classified into a diffuse cutaneous (dcSSc) and a limited cutaneous (IcSSc) subset according to the skin involvement. In order to better understand the vascular, immunological and fibrotic processes of SSc and to guide its treatment, the EULAR Scleroderma Trials And Research (EUSTAR) group was formed in June 2004. Aims and methods: EUSTAR collects prospectively the Minimal Essential Data Set (MEDS) on all sequential patients fulfilling the American College of Rheumatology diagnostic criteria in participating centres. We aimed to characterise demographic, clinical and laboratory characteristics of disease presentation in SSc and analysed EUSTAR baseline visits. Results: In April 2006, a total of 3656 patients (1349 with dcSSc and 2101 with IcSSc) were enrolled in 102 centres and 30 countries. 1330 individuals had autoantibodies against Scl70 and 1106 against anticentromere antibodies. 87% of patients were women. On multivariate analysis, scleroderma subsets (dcSSc vs IcSSc), antibody status and age at onset of Raynaud's phenomenon, but not gender, were found to be independently associated with the prevalence of organ manifestations. Autoantibody status in this analysis was more closely associated with clinical manifestations than were SSc subsets. Conclusion: dcSSc and IcSSc subsets are associated with particular organ manifestations, but in this analysis the clinical distinction seemed to be superseded by an antibody-based classification in predicting some scleroderma complications. The EUSTAR MEDS database facilitates the analysis of clinical patterns in SSc, and contributes to the standardised assessment and monitoring of SSc internationally." }