PT  - JOURNAL ARTICLE
AU  - Justin M. Oldham
AU  - Cathryn T. Lee
AU  - Zhe Wu
AU  - Willis S. Bowman
AU  - Janelle Vu Pugashetti
AU  - Nam Dao
AU  - James Tonkin
AU  - Hasan Seede
AU  - Gabrielle Echt
AU  - Ayodeji Adegunsoye
AU  - Felix Chua
AU  - Toby M. Maher
AU  - Christine K. Garcia
AU  - Mary E. Strek
AU  - Chad A. Newton
AU  - Philip L. Molyneaux
TI  - Lung function trajectory in progressive fibrosing interstitial lung disease
AID  - 10.1183/13993003.01396-2021
DP  - 2022 Jun 01
TA  - European Respiratory Journal
PG  - 2101396
VI  - 59
IP  - 6
4099  - http://erj.ersjournals.com/content/59/6/2101396.short
4100  - http://erj.ersjournals.com/content/59/6/2101396.full
SO  - Eur Respir J2022 Jun 01; 59
AB  - Background Proposed criteria for progressive fibrosing interstitial lung disease (PF-ILD) have been linked to increased mortality risk, but lung function trajectory after satisfying individual criteria remains unknown. Because survival is rarely employed as the primary end-point in therapeutic trials, identifying PF-ILD criteria that best predict subsequent change in forced vital capacity (FVC) could improve clinical trial design.Methods A retrospective, multicentre 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 centres (test cohort) and one UK centre (validation cohort). 1-year change in FVC after satisfying proposed PF-ILD criteria was estimated using joint modelling. Subgroup analyses were performed to determine whether results varied across key subgroups.Results 1227 patients were included, with CTD-ILD predominating. Six out of nine PF-ILD criteria were associated with differential 1-year change in FVC, with radiological 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 radiological 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.Conclusions These findings may inform future clinical trial design and suggest ILD subtype should be taken into consideration when applying PF-ILD criteria.Lung function trajectory varies considerably following ILD diagnosis and after satisfying proposed PF-ILD criteria according to ILD diagnosis. These findings suggest diagnosis should be taken into consideration when designing ILD clinical trials. https://bit.ly/2ZF8C7V