Predictors of disease worsening defined by progression of organ damage in diffuse systemic sclerosis: a European Scleroderma Trials and Research (EUSTAR) analysis. Issue 9 (21st June 2019)

• Record Type: Journal Article
• Authors: Becker, Mike; Graf, Nicole; Sauter, Rafael; Allanore, Yannick; Curram, John; Denton, Christopher P; Khanna, Dinesh; Matucci-Cerinic, Marco; de Oliveira Pena, Janethe; Pope, Janet E; Distler, Oliver
• Other Names: author non-byline.; Matucci-Cerinic Marco author non-byline.; Guiducci Serena author non-byline.; Jaeger Veronika author non-byline.; Bannert Bettina author non-byline.; Lapadula Giovanni author non-byline.; Becvarare Radim author non-byline.; Valentini Gabriele author non-byline.; Siegert Elise author non-byline.; Rednic Simona author non-byline.; Allanore Yannick author non-byline.; Carreira Patricia E author non-byline.; Novak Srdan author non-byline.; Czirják László author non-byline.; Varju Cecilia author non-byline.; Allai Daniela author non-byline.; Cozzi Franco author non-byline.; Rozman Blaz author non-byline.; Gabrielli Armando author non-byline.; Bancel Dominique Farge author non-byline.; Airò Paolo author non-byline.; Balbir-Gurman Alexandra author non-byline.; Pellerito Raffaele author non-byline.; Caramaschi Paola author non-byline.; Damjanov Nemanja author non-byline.; Henes Jörg author non-byline.; Santamaria Vera Ortiz author non-byline.; Heitmann Stefan author non-byline.; Seidel Matthias author non-byline.; Guiducci Serena author non-byline.; Walker Ulrich author non-byline.; Jaeger Veronika author non-byline.; Guiducci Serena author non-byline.; Jaeger Veronika author non-byline.; Bannert Bettina author non-byline.; Lapadula Giovanni author non-byline.; Becvarare Radim author non-byline.; Cutolo Maurizio author non-byline.; Siegert Elise author non-byline.; Rednic Simona author non-byline.; Allanore Yannick author non-byline.; Carreira Patricia E. author non-byline.; Novak Srdan author non-byline.; Czirják László author non-byline.; Varju Cecilia author non-byline.; Allai Daniela author non-byline.; Kucharz Eugene J. author non-byline.; Cozzi Franco author non-byline.; Rozman Blaz author non-byline.; Gabrielli Armando author non-byline.; Bancel Dominique Farge author non-byline.; Airò Paolo author non-byline.; Martinovic Duska author non-byline.; Balbir-Gurman Alexandra author non-byline.; Hunzelmann Nicolas author non-byline.; Pellerito Raffaele author non-byline.; Caramaschi Paola author non-byline.; Damjanov Nemanja author non-byline.; Henes Jörg author non-byline.; Santamaria Vera Ortiz author non-byline.; Heitmann Stefan author non-byline.; Seidel Matthias author non-byline.; Pereira Da Silva José Antonio author non-byline.; Stamenkovic Bojana author non-byline.; Selmi Carlo Francesco author non-byline.; N Denisov Lev author non-byline.; Müller-Ladner Ulf author non-byline.; Engelhart Merete author non-byline.; Hachulla Eric author non-byline.; Riccieri Valeria author non-byline.; Maria Ionescu Ruxandra author non-byline.; Mihai Carina author non-byline.; Sunderkötter Cord author non-byline.; Kuhn Annegret author non-byline.; Schett Georg author non-byline.; Distler Jörg author non-byline.; Meroni Pierluigi author non-byline.; Mouthon Luc author non-byline.; Keyser Filip De author non-byline.; Smith Vanessa author non-byline.; Cantatore Francesco Paolo author non-byline.; Corrado Ada author non-byline.; Ullman Susanne author non-byline.; Iversen Line author non-byline.; Rosa Pozzi Maria author non-byline.; Eyerich Kilian author non-byline.; Hein Rüdiger author non-byline.; Wiland Piotr author non-byline.; Szmyrka-Kaczmarek Magdalena author non-byline.; Sokolik Renata author non-byline.; Morgiel Ewa author non-byline.; Madej Marta author non-byline.; Alegre-Sancho Juan Jose author non-byline.; Krummel-Lorenz Brigitte author non-byline.; Saar Petra author non-byline.; Aringer Martin author non-byline.; Günther Claudia author non-byline.; Anne Erler author non-byline.; Westhovens Rene author non-byline.; De Langhe Ellen author non-byline.; Lenaerts Jan author non-byline.; Anic Branimir author non-byline.; Baresic Marko author non-byline.; Mayer Miroslav author non-byline.; Üprus Maria author non-byline.; Otsa Kati author non-byline.; Yavuz Sule author non-byline.; Cezar Radominski Sebastião author non-byline.; Popa Sergei author non-byline.; Zenone Thierry author non-byline.; Stebbings Simon author non-byline.; Highton John author non-byline.; Mathieu Alessandro author non-byline.; Vacca Alessandra 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.; Amoroso Antonio author non-byline.; Gigante Antonietta author non-byline.; Oksel Fahrettin author non-byline.; Yargucu Figen author non-byline.; Tanaseanu Cristina-Mihaela author non-byline.; Popescu Monica author non-byline.; Dumitrascu Alina author non-byline.; Tiglea Isabela author non-byline.; Foti Rosario author non-byline.; Visalli Elisa author non-byline.; Benenati Alessia author non-byline.; Amato Giorgio author non-byline.; Ancuta Codrina author non-byline.; Chirieac Rodica author non-byline.; Villiger Peter author non-byline.; Adler Sabine author non-byline.; de la Peña Lefebvre Paloma García author non-byline.; Rubio Silvia Rodriguez 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.; Galdo Francesco Del author non-byline.; Venalis Algirdas author non-byline.; Saketkoo Lesley Ann author non-byline.; Kerzberg Eduardo author non-byline.; Montoya Fabiana author non-byline.; Cosentino Vanesa author non-byline.; Limonta Massimiliano author non-byline.; Brucato Antonio Luca author non-byline.; Lupi Elide author non-byline.; Spertini François author non-byline.; Ribi Camillo author non-byline.; Buss Guillaume author non-byline.; Martin Thierry author non-byline.; Guffroy Aurélien author non-byline.; Poindron Vincent author non-byline.; Chung Lori author non-byline.; Schmeiser Tim author non-byline.; Zebryk Pawel author non-byline.; Riso Nuno author non-byline.; Riemekasten Gabriela author non-byline.; Rezus Elena author non-byline.; Puttini Piercarlo Sarzi author non-byline.; Chizzolini Carlo author non-byline.;
• Is Part Of: Annals of the rheumatic diseases. Volume 78:Issue 9(2019)
• Page Start: 1242
• Page End: 1248
• Publication Date: 2019-06-21
• Subjects: systemic sclerosis -- disease worsening -- mortality -- predictive factors; Rheumatism -- Periodicals; 616.723005
• DOI: 10.1136/annrheumdis-2019-215145
• Languages: English
• ISSNs: 0003-4967

Abstract

Abstract : Objectives: Mortality and worsening of organ function are desirable endpoints for clinical trials in systemic sclerosis (SSc). The aim of this study was to identify factors that allow enrichment of patients with these endpoints, in a population of patients from the European Scleroderma Trials and Research group database. Methods: Inclusion criteria were diagnosis of diffuse SSc and follow-up over 12±3 months. Disease worsening/organ progression was fulfilled if any of the following events occurred: new renal crisis; decrease of lung or heart function; new echocardiography-suspected pulmonary hypertension or death. In total, 42 clinical parameters were chosen as predictors for the analysis by using (1) imputation of missing data on the basis of multivariate imputation and (2) least absolute shrinkage and selection operator regression. Results: Of 1451 patients meeting the inclusion criteria, 706 had complete data on outcome parameters and were included in the analysis. Of the 42 outcome predictors, eight remained in the final regression model. There was substantial evidence for a strong association between disease progression and age, active digital ulcer (DU), lung fibrosis, muscle weakness and elevated C-reactive protein (CRP) level. Active DU, CRP elevation, lung fibrosis and muscle weakness were also associated with a significantly shorter time to disease progression. A bootstrap validation step with 10 000 repetitions successfully validated the model. Conclusions: The use of the predictive factors presented here could enable cohort enrichment with patients at risk for overall disease worsening in SSc clinical trials.