Progressive interstitial lung disease in patients with systemic sclerosis-associated interstitial lung disease in the EUSTAR database. Issue 2 (28th September 2020)

• Record Type: Journal Article
• Authors: Hoffmann-Vold, Anna-Maria; Allanore, Yannick; Alves, Margarida; Brunborg, Cathrine; Airó, Paolo; Ananieva, Lidia P; Czirják, László; Guiducci, Serena; Hachulla, Eric; Li, Mengtao; Mihai, Carina; Riemekasten, Gabriela; Sfikakis, Petros P; Kowal-Bielecka, Otylia; Riccardi, Antonella; Distler, Oliver
• Other Names: author non-byline.; Cerinic Marco Matucci author non-byline.; Walker Ulrich author non-byline.; Iannone Florenzo author non-byline.; Becvar Radim author non-byline.; Valentini Gabriele author non-byline.; Siegert Elise author non-byline.; Montecucco C. author non-byline.; Carreira Patricia E. author non-byline.; Chizzolini Carlo author non-byline.; Kucharz Eugene J. author non-byline.; Doria Andrea author non-byline.; Bancel Pr Dominique Farge author non-byline.; Hesselstrand Roger author non-byline.; Balbir-Gurman Alexandra author non-byline.; Pellerito Raffaele author non-byline.; Caimmi Cristian author non-byline.; Denton Christopher author non-byline.; Damjanov Nemanja author non-byline.; Henes Jörg author non-byline.; Heitmann Vera Ortiz-Santamaria; Stefan author non-byline.; Salvador Maria João author non-byline.; Stamenkovic Bojana author non-byline.; Selmi Carlo Francesco author non-byline.; Herrick Ariane author non-byline.; Müller-Ladner Ulf author non-byline.; Engelhart Merete author non-byline.; Riccieri Valeria author non-byline.; Ionescu Ruxandra Maria author non-byline.; Gheorghiu Ana Maria author non-byline.; Sunderkötter Cord author non-byline.; Distler Jörg author non-byline.; Ingegnoli Francesca author non-byline.; Mouthon Luc author non-byline.; Smith Vanessa author non-byline.; Cantatore Francesco Paolo author non-byline.; Ullman Susanne author non-byline.; Pozzi Maria Rosa author non-byline.; Wiland Piotr author non-byline.; Vanthuyne Marie author non-byline.; Krummel-Lorenz Brigitte author non-byline.; Saar Petra author non-byline.; Herrmann Kristine author non-byline.; Langhe Ellen De author non-byline.; Anic Branimir author non-byline.; Baresic Marko author non-byline.; Mayer Miroslav author non-byline.; Yavuz Sule author non-byline.; Müller Carolina de Souza author non-byline.; Zenone Thierry author non-byline.; Mathieu Alessandro author non-byline.; Vacca Alessandra author non-byline.; Solanki Kamal author non-byline.; Rosato Edoardo author non-byline.; Oksel Fahrettin author non-byline.; Yargucu Figen author non-byline.; Tanaseanu Cristina-Mihaela author non-byline.; Foti Rosario author non-byline.; Furst Daniel E. author non-byline.; Villiger Peter author non-byline.; Adler Sabine author non-byline.; de la Peña Lefebvre Paloma García author non-byline.; González Martín Jorge Juan author non-byline.; Litinsky Ira author non-byline.; Galdo Francesco Del author non-byline.; Seskute Goda author non-byline.; Saketkoo Lesley Ann author non-byline.; Kerzberg Eduardo author non-byline.; Castellví Ivan author non-byline.; Spertini François author non-byline.; Hsu Vivien M. author non-byline.; Martin Thierry author non-byline.; Schmeiser Tim author non-byline.; Bernardino Dominik Majewski; Vera author non-byline.; Puttini Piercarlo Sarzi author non-byline.;
• Is Part Of: Annals of the rheumatic diseases. Volume 80:Issue 2(2021)
• Page Start: 219
• Page End: 227
• Publication Date: 2020-09-28
• Subjects: scleroderma -- systemic -- pulmonary fibrosis -- autoimmune diseases; Rheumatism -- Periodicals; 616.723005
• DOI: 10.1136/annrheumdis-2020-217455
• Languages: English
• ISSNs: 0003-4967

Abstract

Abstract : Objectives: To identify overall disease course, progression patterns and risk factors predictive for progressive interstitial lung disease (ILD) in patients with systemic sclerosis-associated ILD (SSc-ILD), using data from the European Scleroderma Trials And Research (EUSTAR) database over long-term follow-up. Methods: Eligible patients with SSc-ILD were registered in the EUSTAR database and had measurements of forced vital capacity (FVC) at baseline and after 12±3 months. Long-term progressive ILD and progression patterns were assessed in patients with multiple FVC measurements. Potential predictors of ILD progression were analysed using multivariable mixed-effect models. Results: 826 patients with SSc-ILD were included. Over 12±3 months, 219 (27%) showed progressive ILD: either moderate (FVC decline 5% to 10%) or significant (FVC decline >10%). A total of 535 (65%) patients had multiple FVC measurements available over mean 5-year follow-up. In each 12-month period, 23% to 27% of SSc-ILD patients showed progressive ILD, but only a minority of patients showed progression in consecutive periods. Most patients with progressive ILD (58%) had a pattern of slow lung function decline, with more periods of stability/improvement than decline, whereas only 8% showed rapid, continuously declining FVC; 178 (33%) experienced no episode of FVC decline. The strongest predictive factors for FVC decline over 5 years were male sex, higher modified Rodnan skin score and reflux/dysphagia symptoms. Conclusion: SSc-ILD shows a heterogeneous and variable disease course, and thus monitoring all patients closely is important. Novel treatment concepts, with treatment initiation before FVC decline occurs, should aim for prevention of progression to avoid irreversible organ damage.