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

  • Hoffmann-Vold, Anna-Maria1
  • Allanore, Yannick2
  • Alves, Margarida3
  • Brunborg, Cathrine4
  • Airó, Paolo5
  • Ananieva, Lidia P6
  • Czirják, László7
  • Guiducci, Serena8
  • Hachulla, Eric9
  • Li, Mengtao10
  • Mihai, Carina11
  • Riemekasten, Gabriela12
  • Sfikakis, Petros P13
  • Kowal-Bielecka, Otylia14
  • Riccardi, Antonella15
  • Distler, Oliver16
  • 1 Department of Rheumatology, Oslo University Hospital, Oslo, Norway. , (Norway)
  • 2 Department of Rheumatology A, Descartes University, APHP, Cochin Hospital, Paris, France. , (France)
  • 3 Boehringer Ingelheim International GmbH, Ingelheim am Rhein, Germany. , (Germany)
  • 4 Oslo Centre for Biostatistics and Epidemiology, Research Support Services, Oslo University Hospital - Rikshospitalet, Oslo, Norway. , (Norway)
  • 5 UO Reumatologia e Immunologia Clinica, Spedali Civili di Brescia, Brescia, Italy. , (Italy)
  • 6 VA Nasonova Institute of Rheumatology, Moscow, Russian Federation. , (Russia)
  • 7 Department of Rheumatology and Immunology, Medical School, University of Pécs, Pécs, Hungary. , (Hungary)
  • 8 Dipartimento di Medicina Sperimentale e Clinica, Università degli Studi di Firenze, Firenze, Italy. , (Italy)
  • 9 Department of Internal Medicine and Clinical Immunology, Hôpital Claude Huriez, University of Lille, Lille, France. , (France)
  • 10 Department of Rheumatology, Peking Union Medical College Hospital (West Campus), Beijing, China. , (China)
  • 11 Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland. , (Switzerland)
  • 12 Department of Rheumatology and Clinical Immunology, University of Lübeck, Lübeck, Germany. , (Germany)
  • 13 Joint Rheumatology Programme, National & Kapodistrian University of Athens Medical School, Athens, Greece. , (Greece)
  • 14 Department of Rheumatology and Internal Medicine, Medical University of Bialystok, Bialystok, Poland. , (Poland)
  • 15 Department of Precision Medicine, Section of Rheumatology, University of Campania Luigi Vanvitelli, Naples, Italy. , (Italy)
  • 16 Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland [email protected] , (Switzerland)
Published Article
Annals of the Rheumatic Diseases
Publication Date
Sep 28, 2020
DOI: 10.1136/annrheumdis-2020-217455
PMID: 32988845


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. © Author(s) (or their employer(s)) 2020. Re-use permitted under CC BY. Published by BMJ.

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