Performance Characteristics for Physiological Measures of Progressive Pulmonary Fibrosis

<b>Rationale:</b> Clinical measures of progressive pulmonary fibrosis (PPF) have been proposed, but their clinical utility remains unclear. <b>Objectives:</b> To determine performance characteristics of lung function-based PPF measures, including new guideline criteria for discriminating clinically relevant outcomes. <b>Methods:</b> A multicenter retrospective cohort analysis was performed to assess the performance characteristics of eight categorical measures of FVC and Dl<sub>CO</sub> decline, together with PPF guideline criteria (requiring two of the following: worsening respiratory symptoms, absolute decline in FVC ⩾5% or Dl<sub>CO</sub> ⩾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 (<i>n</i> = 2,727). The net benefit of the top-performing measures to inform treatment initiation were compared using decision curves. <b>Measurements and Main Results:</b> PPF classified according to relative decline in FVC of ⩾10%, relative decline in Dl<sub>CO</sub> 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 Dl<sub>CO</sub> ⩾15% was similar to PPF guideline criteria across the range of treatment probability thresholds. <b>Conclusions:</b> Classifying PPF by standalone measures of FVC and Dl<sub>CO</sub> decline provides clinical utility similar to PPF guideline criteria. Top-performing physiology-based measures of PPF discriminate outcomes with high specificity but low sensitivity.

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Publication Details

Journal
American Journal of Respiratory and Critical Care Medicine
Published
2025-06-18
DOI
https://doi.org/10.1164/rccm.202501-0317oc
Citations
3
Primary Topic
Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
Type
article
Field-Weighted Citation Impact
4.56
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article

Performance Characteristics for Physiological Measures of Progressive Pulmonary Fibrosis

H. Manganas, Nazanin Nazemi, Justin M. Oldham, Veronica Marcoux et al.
3 citations
American Journal of Respiratory and Critical Care Medicine
Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
4.56
article

Performance Characteristics for Physiological Measures of Progressive Pulmonary Fibrosis

H. Manganas, Nazanin Nazemi, Justin M. Oldham, Veronica Marcoux, Cathryn T. Lee, Janelle Vu Pugashetti, Sahand Ghodrati, Jamie Sheth, Christopher J. Ryerson, Christine Kim Garcia, Kerri A. Johannson, Leda Yazbeck, Nisha Mohan, Yet Hong Khor, Eric S. White, Felix Chua, Chad A. Newton, Philip L. Molyneaux, Ayodeji Adegunsoye, Jennifer M. Wang, Mary E. Strek, Gabrielle Y. Liu, Kevin Flaherty, Nasreen Khalil, Deborah Assayag, Andrew G. Yu, Jolene H. Fisher, Martin Kolb, Julie Morisset, Elizabeth A. Belloli, Ashwatha Thenappan
article en
3 citations

Abstract

<b>Rationale:</b> Clinical measures of progressive pulmonary fibrosis (PPF) have been proposed, but their clinical utility remains unclear. <b>Objectives:</b> To determine performance characteristics of lung function-based PPF measures, including new guideline criteria for discriminating clinically relevant outcomes. <b>Methods:</b> A multicenter retrospective cohort analysis was performed to assess the performance characteristics of eight categorical measures of FVC and Dl<sub>CO</sub> decline, together with PPF guideline criteria (requiring two of the following: worsening respiratory symptoms, absolute decline in FVC ⩾5% or Dl<sub>CO</sub> ⩾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 (<i>n</i> = 2,727). The net benefit of the top-performing measures to inform treatment initiation were compared using decision curves. <b>Measurements and Main Results:</b> PPF classified according to relative decline in FVC of ⩾10%, relative decline in Dl<sub>CO</sub> 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 Dl<sub>CO</sub> ⩾15% was similar to PPF guideline criteria across the range of treatment probability thresholds. <b>Conclusions:</b> Classifying PPF by standalone measures of FVC and Dl<sub>CO</sub> decline provides clinical utility similar to PPF guideline criteria. Top-performing physiology-based measures of PPF discriminate outcomes with high specificity but low sensitivity.

American Journal of Respiratory and Critical Care MedicineVol. 211(10)
University of British Columbia (CA), University of Calgary (CA), University of Toronto (CA), University of Michigan (US), Columbia University Irving Medical Center (US), Royal Brompton & Harefield NHS Foundation Trust (GB), Harefield Hospital (GB), University of Saskatchewan (CA), University of Chicago (US), Monash Alfred Psychiatry Research centre (AU), Royal Brompton Hospital (GB), Michigan United (US), Michigan Medicine (US), Centre Hospitalier de l’Université de Montréal (CA), Austin Health (AU), Pulmonary and Critical Care Associates (US), Imperial College London (GB), McGill University (CA), Monash University (AU), Columbia University (US), University of California, Davis (US), The University of Texas Southwestern Medical Center (US), McMaster University (CA)
Openalex Percentile: Top 5%
Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
4.56
DOISourceOpenAlex
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