Validation of Proposed Criteria for Progressive Pulmonary Fibrosis

Criteria for progressive pulmonary fibrosis (PPF) have been proposed, but their prognostic value beyond categorical decline in FVC remains unclear. To determine whether proposed PPF criteria predict transplant-free survival (TFS) in patients with non-idiopathic pulmonary fibrosis (IPF) forms of inte...

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Bibliographic Details
Published in:American journal of respiratory and critical care medicine 2023-01, Vol.207 (1), p.69-76
Main Authors: Pugashetti, Janelle Vu, Adegunsoye, Ayodeji, Wu, Zhe, 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
Format: Article
Language:English
Subjects:
Classification
Connective Tissue Diseases
Disease Progression
Humans
Idiopathic Pulmonary Fibrosis - complications
Idiopathic Pulmonary Fibrosis - diagnosis
Lung Diseases, Interstitial - diagnosis
Medical diagnosis
Medical prognosis
Medical research
Original
Patients
Prognosis
Pulmonary fibrosis
Retrospective Studies
Survival analysis
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Summary:Criteria for progressive pulmonary fibrosis (PPF) have been proposed, but their prognostic value beyond categorical decline in FVC remains unclear. 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). 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. 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. 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.
ISSN:1073-449X
1535-4970
DOI:10.1164/rccm.202201-0124OC