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- Title
Validation of Proposed Criteria for Progressive Pulmonary Fibrosis.
- Authors
Pugashetti, Janelle Vu; Adegunsoye, Ayodeji; Zhe Wu; Lee, Cathryn T.; Srikrishnan, Anand; Ghodrati, Sahand; Vo, Vivian; Renzoni, Elisabetta A.; Wells, Athol U.; Garcia, Christine Kim; Chua, Felix; Newton, Chad A.; Molyneaux, Philip L.; Oldham, Justin M.
- Abstract
Rationale: Criteria for progressive pulmonary fibrosis (PPF) have been proposed, but their prognostic value beyond categorical decline in FVC remains unclear. Objectives: 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, multicenter cohort analysis was performed. Patients with diagnoses of fibrotic connective tissue disease-associated ILD, fibrotic hypersensitivity pneumonitis, and non-IPF idiopathic interstitial pneumonia from three U.S. centers and one UK center constituted the test and validation cohorts, respectively. Cox proportional hazards regression was used to test the association between 5-year TFS and >10% FVC decline, followed by 13 additional PPF criteria satisfied in the absence of >10% FVC decline. Measurements and Main Results: One thousand three hundred forty-one patients met the 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 U.S. test cohort, with 6 maintaining TFS associations 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 patients with non-IPF ILD at increased risk for death or lung transplantation.
- Subjects
UNITED Kingdom; IDIOPATHIC interstitial pneumonias; PULMONARY fibrosis; INTERSTITIAL lung diseases; HYPERSENSITIVITY pneumonitis; IDIOPATHIC pulmonary fibrosis; LUNG transplantation
- Publication
American Journal of Respiratory & Critical Care Medicine, 2023, Vol 207, Issue 1, p69
- ISSN
1073-449X
- Publication type
Article
- DOI
10.1164/rccm.202201-0124OC