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

EUSTAR Collaborators, Susanne Ullman (Member of study group)

71 Citations (Scopus)

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.

Original languageEnglish
JournalAnnals of the Rheumatic Diseases
Volume80
Issue number2
Pages (from-to)219-227
Number of pages9
ISSN0003-4967
DOIs
Publication statusPublished - Feb 2021

Keywords

  • Adult
  • Databases, Factual
  • Disease Progression
  • Europe/epidemiology
  • Female
  • Humans
  • Lung Diseases, Interstitial/epidemiology
  • Lung/physiopathology
  • Male
  • Middle Aged
  • Prevalence
  • Prospective Studies
  • Risk Factors
  • Scleroderma, Systemic/complications
  • Severity of Illness Index
  • Vital Capacity
  • autoimmune diseases
  • systemic
  • scleroderma
  • pulmonary fibrosis

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