Clinical risk assessment of organ manifestations in systemic sclerosis: A report from the EULAR Scleroderma Trials and Research group database

U. A. Walker, A. Tyndall, L. Czirják, C. Denton, D. Farge-Bancel, O. Kowal-Bielecka, U. Müller-Ladner, C. Bocelli-Tyndall, M. Matucci-Cerinic, Gabriela Riemekasten, Claudia Brückner, Paolo Airó, Mirko Scarsi, Raffaella Scorza, Lorenzo Beretta, Franco Cozzi, Francesco Tiso, M. C. Vonk, F. H.J. Van Den Hoogen, Fredrick M. WigleyLaura Hummers, Tatjana Nevskaya, Lidia Ananieva, Irene Miniati, Nicoletta Tartaglia, Claudia Lomater, Alexandra Balbir-Gurman, Yolanda Braun-Moscovici, Lisa Maria Bambara, Paola Caramaschi, Gabriele Valentini, Luigia Ruocco, Thomas Krieg, Nicolas Hunzelmann, Cecília Varjú, Patricia E. Carriera, Beatriz Joven, Florenzo Iannone, Giovanni Lapadula, André Kahan, Yannick Allanore, Armando Gabrielli, Michele Imperatore, Agneta Scheja, Frank Wollheim, Nemanja Damjanov, Predrag Ostojic, Petra Saar, Ingo H. Tarner, Ina Kötter, Stefano Bombardieri, Laura Bazzichi, Nicoletta Del Papa, Denise P. Comina, Andrea Lo Monaco, Renato La Corte, Eric Hachulla, David Launay, Oliver Distler, Adrian Ciurea, Stanislaw Sierakowski, Holly Mitchell, Richard M. Silver, Dorota Krasowska, Malgorzata Michalska-Jakubus, Mohammed Tikly, Nazrana Aboo, Margitta Worm, Pascal Klaus, Jozef Rovenský, Olga Lukáčová, Blaz Rozman, Alenka Sipek, Paulo Clemente-Coelho, Yehuda Shoenfeld, Pnina Langewitch, A. P. Da Silva José, M. J. Salvador, Annegret Kuhn, Gunilla Erdmann, Radim Bečvář, Elke Friedl, Winfried Graninger, Valeria Riccieri, Roberto Caporali, Carlomaurizio Montecucco, P. Vlachoyiannopoulos, Meike Distler, Kristian Reich, Maria Majdan, Ewa Wielosz, Simona Rednic, Jacob M. Van Laar, Stefan Heitmann, Andreas Bruckner, Andrea Himsel, Julia Riemann, Rotraud Meyringer, Adelheid Müller, Duska Martinovic, Mislav Radic, Michael Sticherling, Zoltan Szekanecz, Gabriella Szücs, Roberto Giacomelli, Alessandra Marrelli, Bojana Stamenkovic, Aleksandra Stankovic, Martin Aringer, Josef S. Smolen, Eugene J. Kucharz, Anna T. Kotulska, Stefania Jablonska, Maria Blasczik, Jae Bum Jun, Carmel Mallia, Bernard Coleiro, Vera Ortiz Santamaria, Ralf Hinrichs, Henrik Nielsen, Roberta Cossutta, Ruxandra Ionescu, Daniela Opris, Kerstin Steinbrink, Boris Grundt, Gianluigi Bajocchi, Štork Jiří, Paloma García De La Peña Lefebvre, Antonio C. Zea Mendoza, Camillo Ribi, Carlo Chizzolini, Margaret Wisłowska, Srdan Novak, Francesco Indiveri, Søren Jacobsen, Per Brown Frandsen, I. Zimmermann Gorska, Jane Tore Gran, Øyvind Midtvedt, Filipa Oliveira Ramos, Ljubinka Damjanovska Rajcevska, Georgi Bozinovski, Dieter Schöffel, Cord Sunderkötter, Markus Böhm, Jadranka Morović-Vergles, Melanie Ivana Čulo, Maurizio Cutolo, Alberto Sulli, Chris T. Derk, Sergio A. Jimenez, Panagiota Siakka, Klaus Søndergaard, Kristian Stengaard-Pedersen, Jean Cabane, Kiet Phong Tiev, Carina Mihai, Roxana Sfrent-Cornateanu, Michael Jendro, Piia Tuvik, Marco Antivalle, Giovanna Randisi, Matthias Seidel, Ricarda Clarenbach, Ismail Simsek, Ayhan Dinc, Murat Inanc, Monica Sinziana Capraru, Dorin Capraru, Inmaculada Bañegil, Jutta Richter, Saad Alhasani, Ivan Földvari, Sandra Pinto, Filipe Brandão, Antonio Juan Mas

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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.

Original languageEnglish
Pages (from-to)754-763
Number of pages10
JournalAnnals of the rheumatic diseases
Volume66
Issue number6
DOIs
Publication statusPublished - Jun 2007

ASJC Scopus subject areas

  • Immunology and Allergy
  • Rheumatology
  • Immunology
  • Biochemistry, Genetics and Molecular Biology(all)

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    Walker, U. A., Tyndall, A., Czirják, L., Denton, C., Farge-Bancel, D., Kowal-Bielecka, O., Müller-Ladner, U., Bocelli-Tyndall, C., Matucci-Cerinic, M., Riemekasten, G., Brückner, C., Airó, P., Scarsi, M., Scorza, R., Beretta, L., Cozzi, F., Tiso, F., Vonk, M. C., Van Den Hoogen, F. H. J., ... Mas, A. J. (2007). Clinical risk assessment of organ manifestations in systemic sclerosis: A report from the EULAR Scleroderma Trials and Research group database. Annals of the rheumatic diseases, 66(6), 754-763. https://doi.org/10.1136/ard.2006.062901