Lung function trajectory in progressive fibrosing interstitial lung disease

Justin M. Oldham, Cathryn T. Lee, Zhe Wu, Willis S. Bowman, Janelle Vu Pugashetti, Nam Dao, James Tonkin, Hasan Seede, Gabrielle Echt, Ayodeji Adegunsoye, Felix Chua, Toby M. Maher, Christine K Garcia, Mary E. Strek, Chad A. Newton, Philip L. Molyneaux

Research output: Contribution to journalArticlepeer-review

4 Scopus citations


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.

Original languageEnglish (US)
JournalThe European respiratory journal
Issue number6
StatePublished - Jun 1 2022

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine


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