BibTex format

author = {Pugashetti, JV and Adegunsoye, A and Wu, Z and Lee, CT and Srikrishnan, A and Ghodrati, S and Vo, V and Renzoni, EA and Wells, AU and Garcia, CK and Chua, F and Newton, CA and Molyneaux, PL and Oldham, JM},
doi = {10.1164/rccm.202201-0124OC},
journal = {American Journal of Respiratory and Critical Care Medicine},
title = {Validation of proposed criteria for progressive pulmonary fibrosis.},
url = {},
year = {2022}

RIS format (EndNote, RefMan)

AB - 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.
AU - Pugashetti,JV
AU - Adegunsoye,A
AU - Wu,Z
AU - Lee,CT
AU - Srikrishnan,A
AU - Ghodrati,S
AU - Vo,V
AU - Renzoni,EA
AU - Wells,AU
AU - Garcia,CK
AU - Chua,F
AU - Newton,CA
AU - Molyneaux,PL
AU - Oldham,JM
DO - 10.1164/rccm.202201-0124OC
PY - 2022///
SN - 1073-449X
TI - Validation of proposed criteria for progressive pulmonary fibrosis.
T2 - American Journal of Respiratory and Critical Care Medicine
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