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

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 (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), pp. 219-227. BMJ Publishing Group 10.1136/annrheumdis-2020-217455

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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.


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.


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.


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.

Item Type:

Journal Article (Original Article)


04 Faculty of Medicine > Department of Dermatology, Urology, Rheumatology, Nephrology, Osteoporosis (DURN) > Clinic of Rheumatology, Clinical Immunology and Allergology


600 Technology > 610 Medicine & health




BMJ Publishing Group




Marlise Bühler Zimmermann

Date Deposited:

04 Jan 2021 16:58

Last Modified:

17 Mar 2021 04:08

Publisher DOI:


PubMed ID:


Additional Information:

EUSTAR collaborators: Peter Villiger

Uncontrolled Keywords:

autoimmune diseases pulmonary fibrosis scleroderma systemic




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