Progressive interstitial lung disease in patients with systemic sclerosis-associated interstitial lung disease in the EUSTAR database.

Hoffmann-Vold AM, Allanore Y, Alves M, Brunborg C, Airo P, Ananieva LP, Czirjak L, Guiducci S, Hachulla E, Li M, Mihai C, Riemekasten G, Sfikakis PP, Kowal-Bielecka O, Riccardi A, Distler O (2021)

Publication Type: Journal article

Publication year: 2021

Journal

Book Volume: 80

Pages Range: 219-227

Journal Issue: 2

DOI: 10.1136/annrheumdis-2020-217455

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.

Involved external institutions

Oslo University Hospital / Oslo Universitetssykehus Rikshospitalet Norway (NO) University of Paris 5 - René Descartes / Université Paris V René Descartes France (FR) Boehringer Ingelheim Pharma GmbH & Co. KG Germany (DE) Azienda Socio Sanitaria Territoriale degli Spedali Civili di Brescia Italy (IT) V.A. Nasonova Research Institute of Rheumatology Russian Federation (RU) University of Pécs / Pécsi Tudományegyetem Hungary (HU) Università degli Studi di Firenze / University of Florence Italy (IT) LILLE 1 University - Science and Technology France (FR) Peking Union Medical College / 北京协和医学院 China (CN) Universitätsspital Zürich (USZ) Switzerland (CH) Universität zu Lübeck Germany (DE) National and Kapodistrian University of Athens Greece (GR) University of Białystok Poland (PL) Università degli studi della Campania Luigi Vanvitelli Italy (IT)

How to cite

APA:

Hoffmann-Vold, A.-M., Allanore, Y., Alves, M., Brunborg, C., Airo, P., Ananieva, L.P.,... Distler, O. (2021). Progressive interstitial lung disease in patients with systemic sclerosis-associated interstitial lung disease in the EUSTAR database. Annals of the Rheumatic Diseases, 80(2), 219-227. https://doi.org/10.1136/annrheumdis-2020-217455

MLA:

Hoffmann-Vold, Anna-Maria, et al. "Progressive interstitial lung disease in patients with systemic sclerosis-associated interstitial lung disease in the EUSTAR database." Annals of the Rheumatic Diseases 80.2 (2021): 219-227.

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