Faculty, Staff and Student Publications

Language

English

Publication Date

10-1-2025

Journal

American Journal of Respiratory and Critical Care Medicine

DOI

10.1164/rccm.202501-0317OC

PMID

40530983

PMCID

PMC12525792

PubMedCentral® Posted Date

10-16-2025

PubMedCentral® Full Text Version

Author MSS

Abstract

Rationale: Clinical measures of progressive pulmonary fibrosis (PPF) have been proposed, but their clinical utility remains unclear.

Objectives: To determine performance characteristics of lung function-based PPF measures, including new guideline criteria for discriminating clinically relevant outcomes.

Methods: A multicenter retrospective cohort analysis was performed to assess the performance characteristics of eight categorical measures of FVC and DlCO decline, together with PPF guideline criteria (requiring two of the following: worsening respiratory symptoms, absolute decline in FVC ⩾5% or DlCO ⩾15%, or radiological progression) for discriminating 2-year death or lung transplant among patients fibrotic interstitial lung disease from the United States, United Kingdom, and Canada (n = 2,727). The net benefit of the top-performing measures to inform treatment initiation were compared using decision curves.

Measurements and Main Results: PPF classified according to relative decline in FVC of ⩾10%, relative decline in DlCO of ⩾15%, and PPF guideline criteria displayed the best overall test performance, with area under the receiver operating characteristic curves of 0.67-0.68. Specificity was higher than sensitivity for all evaluated measures, with relative measures of lung function decline outperforming absolute measures. The net benefit of standalone relative decline in FVC ⩾10% and DlCO ⩾15% was similar to PPF guideline criteria across the range of treatment probability thresholds.

Conclusions: Classifying PPF by standalone measures of FVC and DlCO decline provides clinical utility similar to PPF guideline criteria. Top-performing physiology-based measures of PPF discriminate outcomes with high specificity but low sensitivity.

Keywords

Aged, Female, Humans, Male, Middle Aged, Canada, Disease Progression, Pulmonary Fibrosis, Respiratory Function Tests, Retrospective Studies, ROC Curve, United Kingdom, United States, Vital Capacity, Interstitial Lung Disease, Idiopathic Pulmonary Fibrosis, Progressive Fibrosing Interstitial Lung Disease, Progressive Pulmonary Fibrosis, Test Performance Characteristics

Published Open-Access

yes

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