Prediction of worsening of skin fibrosis in patients with diffuse cutaneous systemic sclerosis using the EUSTAR database

作者：Britta Maurer, Nicole Graf, Beat A. Michel, Ulf Müller‐Ladner, László Czirják, Christopher P. Denton, Alan Tyndall, Carola Metzig, Vivian Lanius, Dinesh Khanna, Oliver Distler, Ingo H Tarner, Marco Matucci Cerinic, Serena Guiducci, Ulrich Walker, Giovanni Lapadula, Florenzo Iannone, Radim Becvar, Stanislaw Sierakowsky, Otylia Kowal Bielecka, Maurizio Cutolo, Alberto Sulli, Gabriele Valentini, Giovanna Cuomo, Serena Vettori, Gabriele Riemekasten, Simona Rednic, Ileana Nicoara, André Kahan, Yannick Allanore, P Vlachoyiannopoulos, C Montecucco, Roberto Caporali, Patricia E Carreira, Srdan Novak, Cecilia Varju, Carlo Chizzolini, Eugene J Kucharz, Anna Kotulska, Magdalena Kopec-Medrek, Malgorzata Widuchowska, Franco Cozzi, Blaz Rozman, Carmel Mallia, Bernard Coleiro, Armando Gabrielli, Dominique Farge, Adrian Hij, Paolo Airò, Roger Hesselstrand, Agneta Scheja, Frank Wollheim, Duska Martinovic, Alexandra Balbir Gurman, Yolanda Braun-Moscovici, M Govoni, Andrea Lo Monaco, Nicolas Hunzelmann, Raffaele Pellerito, Lisa Maria Bambara, Paola Caramaschi, Carol Black, Nemanja Damjanov, Vera Ortiz Santamaria, Stefan Heitmann, Dorota Krasowska, Matthias Seidel, Mara Oleszowsky, Harald Burkhardt, Andrea Himsel, Maria J Salvador, Bojana Stamenkovic, Aleksandra Stankovic, Mohammed Tikly, Maya N Starovoytova, Lidia P Ananieva, Raffaella Scorza, Merete Engelhart, Gitte Strauss, Henrik Nielsen, Kirsten Damgaard, Gabriella Szücs, Antonio Zea Mendoza, Carlos de la Puente Buijdos, Walter A. Sifuentes Giraldo, Øyvind Midtvedt, Torhild Garen, Eric Hachulla, David Launay, Guido Valesini, Valeria Riccieri, Ruxandra Maria Ionescu, Daniela Opris, Laura Groseanu, Fredrick M Wigley, Carmen M Mihai, Roxana Sfrent Cornateanu, Razvan Ionitescu, Ana Maria Gherghe, Marilena Gorga, Rucsandra Dobrota, Mihai Bojinca, Georg Schett, Jörg HW Distler, Pierluigi Meroni, Silvana Zeni, Luc Mouthon, Filip De Keyser, Francesco P Cantatore, Ada Corrado, Susanne Ullman, Line Iversen, Maria R Pozzi, Kilian Eyerich, Rüdiger Hein, Elisabeth Knott, Jacek Szechinski, Piotr Wiland, Magdalena Szmyrka-Kaczmarek, Renata Sokolik, Ewa Morgiel, Brigitte Krummel-Lorenz, Petra Saar, Martin Aringer, Claudia Günther, Branimir Anic, Marko Baresic, Miroslav Mayer, Sebastião C Radominski, Carolina de Souza Müller, Valderílio F Azevedo, Svetlana Agachi, Liliana Groppa, Lealea Chiaburu, Eugen Russu, Thierry Zenone, John Highton, Lisa Stamp, Peter Chapman, John O'Donnell, Kamal Solanki, Alan Doube, Douglas Veale, Marie O’Rourke, Esthela Loyo, Mengtao Li, Edoardo Rosato, Simonetta Pisarri, Cristina-Mihaela Tanaseanu, Monica Popescu, Alina Dumitrascu, Isabela Tiglea, Rodica Chirieac, Codrina Ancuta, Daniel E Furst, Suzanne Kafaja, Paloma García de la Peña Lefebvre, Silvia Rodriguez Rubio, Marta Valero Exposito, Jean Sibilia, Emmanuel Chatelus, Jacques Eric Gottenberg, Hélène Chifflot, Ira Litinsky, Algirdas Venalis, Irena Butrimiene, Paulius Venalis, Rita Rugiene, Diana Karpec, Eduardo Kerzberg, Fabiana Montoya, Vanesa Cosentino · 发表于：Annals of the Rheumatic Diseases · 年份：2014 · DOI：10.1136/annrheumdis-2014-205226 · 被引用次数：128 · 研究领域：Systemic Sclerosis and Related Diseases、Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis、Rheumatoid Arthritis Research and Therapies

OBJECTIVES: To identify predictive parameters for the progression of skin fibrosis within 1 year in patients with diffuse cutaneous SSc (dcSSc). METHODS: An observational study using the EUSTAR database was performed. Inclusion criteria were dcSSc, American College of Rheumatology (ACR) criteria fulfilled, modified Rodnan skin score (MRSS) ≥7 at baseline visit, valid data for MRSS at 2nd visit, and available follow-up of 12±2 months. Worsening of skin fibrosis was defined as increase in MRSS >5 points and ≥25% from baseline to 2nd visit. In the univariate analysis, patients with progressive fibrosis were compared with non-progressors, and predictive markers with p<0.2 were included in the logistic regression analysis. The prediction models were then validated in a second cohort. RESULTS: A total of 637 dcSSc patients were eligible. Univariate analyses identified joint synovitis, short disease duration (≤15 months), short disease duration in females/patients without creatine kinase (CK) elevation, low baseline MRSS (≤22/51), and absence of oesophageal symptoms as potential predictors for progressive skin fibrosis. In the multivariate analysis, by employing combinations of the predictors, 17 models with varying prediction success were generated, allowing cohort enrichment from 9.7% progressive patients in the whole cohort to 44.4% in the optimised enrichment cohort. Using a second validation cohort of 188 dcSSc patients, short disease duration, low baseline MRSS and joint synov...