Abstract
Background: Some patients with systemic sclerosis-associated interstitial lung disease (SSc-ILD) develop progressive ILD, but disease course and outcome are unknown.
Objectives: To assess frequency, disease course and mortality of progressive ILD in the European Scleroderma Trials and Research group (EUSTAR) database.
Methods: Adult patients fulfilling SSc classification criteria, registered since 2010, with ILD on high- resolution computed tomography, serial lung function and disease duration recordings, were eligible. Progressive ILD was defined as: significant (forced vital capacity [FVC] decline >10% or 5-10% with diffusing capacity for carbon monoxide decline ≥15%); moderate (FVC decline 5-10%); or stable (<5% FVC change). Lung fibrosis changes were assessed from baseline to 12±3 months; disease course, factors associated with further ILD progression and mortality were determined during 5-yr follow-up.
Results: Of 826 patients included, serial lung function data (median follow-up 5 yrs) were available in 411 (49.8%); 124/411 (30.2%) had stable lung function throughout the 5 yrs. In 128/411 (31%) with initial progressive ILD at 12±3 months, 59/128 (46.1%) showed further progression. Baseline FVC, baseline extent of skin fibrosis and disease duration were significantly associated with further ILD progression. 85/826 (10.3%) patients died, 11/124 (8.9%) with stable ILD. 10/59 (16.9%) with progressive ILD at 12±3 months and further progression during follow-up died vs 4/69 (5.8%) with stable ILD after initial progression (P=0.004).
Conclusion: While not all patients with SSc-ILD show progression, prevention of further ILD progression may improve survival.
Funding: Boehringer Ingelheim
Footnotes
Cite this article as: European Respiratory Journal 2019; 54: Suppl. 63, OA3598.
This is an ERS International Congress abstract. No full-text version is available. Further material to accompany this abstract may be available at www.ers-education.org (ERS member access only).
- Copyright ©the authors 2019