Validation of proposed criteria for progressive pulmonary fibrosis.
File(s)PF-ILD Surv Ms_R3_Clean.docx (194.11 KB)
Accepted version
Author(s)
Type
Journal Article
Abstract
RATIONALE: Criteria for progressive pulmonary fibrosis (PPF) have been proposed, but their prognostic value beyond categorical decline in forced vital capacity (FVC) remains unclear. OBJECTIVE: To determine whether proposed PPF criteria predict transplant-free survival (TFS) in patients with non-idiopathic pulmonary fibrosis (IPF) forms of interstitial lung disease (ILD). METHODS: A retrospective, multi-center cohort analysis was performed. Patients diagnosed with fibrotic connective tissue disease associated ILD, fibrotic hypersensitivity pneumonitis and non-IPF idiopathic interstitial pneumonia from three US centers and one UK center comprised test and validation cohorts, respectively. Cox proportional hazards regression was used to test the association between five-year TFS and 10% FVC decline, followed by thirteen additional PPF criteria satisfied in the absence of >=10% FVC decline. MAIN RESULTS: One thousand three hundred forty-one patients met inclusion criteria. A >=10% relative FVC decline was the strongest predictor of reduced TFS and showed consistent TFS association across cohorts, ILD subtypes and treatment groups, resulting in a phenotype that closely resembled IPF. Ten additional PPF criteria satisfied in the absence of >=10% relative FVC decline were also associated with reduced TFS in the US test cohort, with six maintaining TFS association in the UK validation cohort. Validated PPF criteria requiring a combination of physiologic, radiologic, and symptomatic worsening performed similarly to their stand-alone components but captured a smaller number of patients. CONCLUSIONS: An FVC decline of >=10% and six additional PPF criteria satisfied in the absence of such decline identify non-IPF ILD patients at increased risk of death or lung transplant.
Date Issued
2022-08-09
Date Acceptance
2022-08-09
Citation
American Journal of Respiratory and Critical Care Medicine, 2022, 207 (1), pp.69-76
ISSN
1073-449X
Publisher
American Thoracic Society
Start Page
69
End Page
76
Journal / Book Title
American Journal of Respiratory and Critical Care Medicine
Volume
207
Issue
1
Copyright Statement
Copyright © 2023 by the American Thoracic Society. Originally Published in: [Authors].American Journal of Respiratory and Critical Care Medicine. DOI: https://doi.org/10.1164/rccm.202201-0124OC
Identifier
https://www.ncbi.nlm.nih.gov/pubmed/35943866
Subjects
idiopathic interstitial pneumonia
idiopathic pulmonary fibrosis
interstitial lung disease
progressive fibrosing interstitial lung disease
progressive pulmonary fibrosis
Publication Status
Published online
Coverage Spatial
United States
Date Publish Online
2022-08-09