Lung function trajectory in progressive fibrosing interstitial lung disease
File(s)PF-ILD Manuscript_Final_Clean.docx (833.83 KB)
Accepted version
Author(s)
Type
Journal Article
Abstract
Proposed criteria for progressive fibrosing interstitial lung disease (PF-ILD) have been linked to increased mortality risk, but lung function trajectory after satisfying individual criterion remains unknown. Because survival is rarely employed as the primary endpoint in therapeutic trials, identifying PF-ILD criteria that best predict subsequent change in forced vital capacity (FVC) could improve clinical trial design. A retrospective, multi-center longitudinal cohort analysis was performed in consecutive patients with fibrotic connective tissue disease-associated ILD (CTD-ILD), chronic hypersensitivity pneumonitis and idiopathic interstitial pneumonia at three US centers (test cohort) and one UK center (validation cohort). One-year change in FVC after satisfying proposed PF-ILD criteria was estimated using joint modeling. Subgroup analyses were performed to determine whether results varied across key subgroups. One thousand two hundred twenty-seven patients were included, with CTD-ILD predominating. Six of nine PF-ILD criteria were associated with differential one-year change in FVC, with radiologic progression of fibrosis, alone and in combination with other features, associated with the largest subsequent decline in FVC. Findings varied significantly by ILD subtype, with CTD-ILD demonstrating little change in FVC after satisfying most PF-ILD criteria, while other ILDs showed significantly larger changes. Findings did not vary after stratification by radiologic pattern or exposure to immunosuppressant therapy. Near-term change in FVC after satisfying proposed PF-ILD criteria was heterogeneous depending on the criterion assessed and was strongly influenced by ILD subtype. These findings may inform future clinical trial design and suggest ILD subtype should be taken into consideration when applying PF-ILD criteria.
Date Acceptance
2021-10-20
Citation
European Respiratory Journal, 59 (6)
ISSN
0903-1936
Publisher
European Respiratory Society
Journal / Book Title
European Respiratory Journal
Volume
59
Issue
6
Copyright Statement
© The authors 2021. For reproduction rights and permissions contact permissions@ersnet.org
Sponsor
Action for Pulmonary Fibrosis
National Institute for Health Research
British Lung Foundation
Identifier
https://erj.ersjournals.com/content/early/2021/10/28/13993003.01396-2021
Grant Number
n/a
CS-2013-13-017
C17-3
Subjects
Respiratory System
11 Medical and Health Sciences
Publication Status
Published online
Date Publish Online
2021-11-04