Title :

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

Author(s) :

Hoffmann-Vold, Anna-Maria [Auteur]
Oslo University Hospital [Oslo]
Allanore, Yannick [Auteur]
Hôpital Cochin [AP-HP]
Alves, Margarida [Auteur]
Boehringer Ingelheim International GmbH
Brunborg, Cathrine [Auteur]
Oslo University Hospital [Oslo]
Airo, Paolo [Auteur]
Civic Hospital of Brescia
Ananieva, Lidia P. [Auteur]
Czirjak, Laszlo [Auteur]
University of Pecs
Guiducci, Serena [Auteur]
Università degli Studi di Firenze = University of Florence = Université de Florence [UniFI]
Hachulla, Eric [Auteur]
Centre Hospitalier Régional Universitaire [CHU Lille] [CHRU Lille]
Li, Mengtao [Auteur]
Peking Union Medical College Hospital [Beijing] [PUMCH]
Mihai, Carina [Auteur]
University hospital of Zurich [Zurich]
Riemekasten, Gabriela [Auteur]
Universität zu Lübeck = University of Lübeck [Lübeck]
Sfikakis, Petros P. [Auteur]
University of Athens Medical School [Athens]
Kowal-Bielecka, Otylia [Auteur]
Medical University of Białystok [MUB]
Riccardi, Antonella [Auteur]
Università degli studi della Campania "Luigi Vanvitelli" = University of the Study of Campania Luigi Vanvitelli
Distler, Oliver [Auteur]
University hospital of Zurich [Zurich]

Journal title :

Annals of the Rheumatic Diseases

Abbreviated title :

Ann. Rheum. Dis.

Publication date :

2020-09-28

ISSN :

1468-2060

HAL domain(s) :

Sciences du Vivant [q-bio]

English abstract : [en]

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 ...
Show more >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.Show less >

Language :

Anglais

Audience :

Internationale

Popular science :

Non

Administrative institution(s) :

CHU Lille
Inserm
Université de Lille

Collections :

• Institut de Recherche Translationnelle sur l'Inflammation (INFINITE) - U1286

Submission date :

2021-07-06T12:46:05Z
2024-01-31T11:05:50Z