Prediction of worsening of skin fibrosis in patients with diffuse cutaneous systemic sclerosis using the EUSTAR database. Issue 6 (30th June 2014)

• Record Type: Journal Article
• Authors: Maurer, Britta; Graf, Nicole; Michel, Beat A; Müller-Ladner, Ulf; Czirják, László; Denton, Christopher P; Tyndall, Alan; Metzig, Carola; Lanius, Vivian; Khanna, Dinesh; Distler, Oliver
• Other Names: Tarner Ingo H author non-byline.; Cerinic Marco Matucci author non-byline.; Guiducci Serena author non-byline.; Walker Ulrich author non-byline.; Lapadula Giovanni author non-byline.; Iannone Florenzo author non-byline.; Becvar Radim author non-byline.; Sierakowsky Stanislaw author non-byline.; Bielecka Otylia Kowal author non-byline.; Cutolo Maurizio author non-byline.; Sulli Alberto author non-byline.; Valentini Gabriele author non-byline.; Cuomo Giovanna author non-byline.; Vettori Serena author non-byline.; Riemekasten Gabriele author non-byline.; Rednic Simona author non-byline.; Nicoara Ileana author non-byline.; Kahan André author non-byline.; Allanore Yannick author non-byline.; Vlachoyiannopoulos P author non-byline.; Montecucco C author non-byline.; Caporali Roberto author non-byline.; Carreira Patricia E author non-byline.; Novak Srdan author non-byline.; Varju Cecilia author non-byline.; Chizzolini Carlo author non-byline.; Kucharz Eugene J author non-byline.; Kotulska Anna author non-byline.; Kopec-Medrek Magdalena author non-byline.; Widuchowska Malgorzata author non-byline.; Cozzi Franco author non-byline.; Rozman Blaz author non-byline.; Mallia Carmel author non-byline.; Coleiro Bernard author non-byline.; Gabrielli Armando author non-byline.; Farge Dominique author non-byline.; Hij Adrian author non-byline.; Airò Paolo author non-byline.; Hesselstrand Roger author non-byline.; Scheja Agneta author non-byline.; Wollheim Frank author non-byline.; Martinovic Duska author non-byline.; Gurman Alexandra Balbir author non-byline.; Braun-Moscovici Yolanda author non-byline.; Govoni M author non-byline.; Monaco Andrea Lo author non-byline.; Hunzelmann Nicolas author non-byline.; Pellerito Raffaele author non-byline.; Bambara Lisa Maria author non-byline.; Caramaschi Paola author non-byline.; Black Carol author non-byline.; Damjanov Nemanja author non-byline.; Santamaria Vera Ortiz author non-byline.; Heitmann Stefan author non-byline.; Krasowska Dorota author non-byline.; Seidel Matthias author non-byline.; Oleszowsky Mara author non-byline.; Burkhardt Harald author non-byline.; Himsel Andrea author non-byline.; Salvador Maria J author non-byline.; Stamenkovic Bojana author non-byline.; Stankovic Aleksandra author non-byline.; Tikly Mohammed author non-byline.; Starovoytova Maya N author non-byline.; Ananieva Lidia P author non-byline.; Scorza Raffaella author non-byline.; Engelhart Merete author non-byline.; Strauss Gitte author non-byline.; Nielsen Henrik author non-byline.; Damgaard Kirsten author non-byline.; Szücs Gabriella author non-byline.; Mendoza Antonio Zea author non-byline.; Buijdos Carlos de la Puente author non-byline.; Sifuentes Giraldo Walter A. author non-byline.; Midtvedt Øyvind author non-byline.; Garen Torhild author non-byline.; Hachulla Eric author non-byline.; Launay David author non-byline.; Valesini Guido author non-byline.; Riccieri Valeria author non-byline.; Ionescu Ruxandra Maria author non-byline.; Opris Daniela author non-byline.; Groseanu Laura author non-byline.; Wigley Fredrick M author non-byline.; Mihai Carmen M author non-byline.; Cornateanu Roxana Sfrent author non-byline.; Ionitescu Razvan author non-byline.; Gherghe Ana Maria author non-byline.; Gorga Marilena author non-byline.; Dobrota Rucsandra author non-byline.; Bojinca Mihai author non-byline.; Schett Georg author non-byline.; Distler Jörg HW author non-byline.; Meroni Pierluigi author non-byline.; Zeni Silvana author non-byline.; Mouthon Luc author non-byline.; Keyser Filip De author non-byline.; Cantatore Francesco P author non-byline.; Corrado Ada author non-byline.; Ullman Susanne author non-byline.; Iversen Line author non-byline.; Pozzi Maria R author non-byline.; Eyerich Kilian author non-byline.; Hein Rüdiger author non-byline.; Knott Elisabeth author non-byline.; Szechinski Jacek author non-byline.; Wiland Piotr author non-byline.; Szmyrka-Kaczmarek Magdalena author non-byline.; Sokolik Renata author non-byline.; Morgiel Ewa author non-byline.; Krummel-Lorenz Brigitte author non-byline.; Saar Petra author non-byline.; Aringer Martin author non-byline.; Günther Claudia author non-byline.; Anic Branimir author non-byline.; Baresic Marko author non-byline.; Mayer Miroslav author non-byline.; Radominski Sebastião C author non-byline.; Müller Carolina de Souza author non-byline.; Azevedo Valderílio F author non-byline.; Agachi Svetlana author non-byline.; Groppa Liliana author non-byline.; Chiaburu Lealea author non-byline.; Russu Eugen author non-byline.; Zenone Thierry author non-byline.; Highton John author non-byline.; Stamp Lisa author non-byline.; Chapman Peter author non-byline.; O'Donnell John author non-byline.; Solanki Kamal author non-byline.; Doube Alan author non-byline.; Veale Douglas author non-byline.; O'Rourke Marie author non-byline.; Loyo Esthela author non-byline.; Li Mengtao author non-byline.; Rosato Edoardo author non-byline.; Pisarri Simonetta author non-byline.; Tanaseanu Cristina-Mihaela author non-byline.; Popescu Monica author non-byline.; Dumitrascu Alina author non-byline.; Tiglea Isabela author non-byline.; Chirieac Rodica author non-byline.; Ancuta Codrina author non-byline.; E Furst Daniel author non-byline.; Kafaja Suzanne author non-byline.; Lefebvre Paloma García de la Peña author non-byline.; Rubio Silvia Rodriguez author non-byline.; Exposito Marta Valero author non-byline.; Sibilia Jean author non-byline.; Chatelus Emmanuel author non-byline.; Gottenberg Jacques Eric author non-byline.; Chifflot Hélène author non-byline.; Litinsky Ira author non-byline.; Venalis Algirdas author non-byline.; Butrimiene Irena author non-byline.; Venalis Paulius author non-byline.; Rugiene Rita author non-byline.; Karpec Diana author non-byline.; Kerzberg Eduardo author non-byline.; Montoya Fabiana author non-byline.; Cosentino Vanesa author non-byline.;
• Is Part Of: Annals of the rheumatic diseases. Volume 74:Issue 6(2015)
• Page Start: 1124
• Page End: 1131
• Publication Date: 2014-06-30
• Subjects: Systemic Sclerosis -- Outcomes Research -- Qualitative Research; Rheumatism -- Periodicals; 616.723005
• DOI: 10.1136/annrheumdis-2014-205226
• Languages: English
• ISSNs: 0003-4967

Abstract

Abstract : 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 synovitis were confirmed as independent predictors of progressive skin fibrosis within 1 year resulting in a 4.5-fold increased prediction success rate. Conclusions: Our study provides novel, evidence-based criteria for the enrichment of dcSSc cohorts with patients who experience worsening of skin fibrosis which allows improved clinical trial design.