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Related Experiment Video

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Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
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Lung function trajectory in progressive fibrosing interstitial lung disease.

Justin M Oldham1,2, Cathryn T Lee3,2, Zhe Wu4,5

  • 1Division of Pulmonary, Critical Care and Sleep Medicine, University of California at Davis, Sacramento, CA, USA joldham@ucdavis.edu.

The European Respiratory Journal
|November 5, 2021
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Identifying progressive fibrosing interstitial lung disease (PF-ILD) criteria that predict forced vital capacity (FVC) decline is crucial. The study found that specific PF-ILD criteria, particularly radiological progression, significantly impact FVC change, varying by interstitial lung disease (ILD) subtype.

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Area of Science:

  • Pulmonary Medicine
  • Clinical Research
  • Interstitial Lung Disease

Background:

  • Proposed criteria for progressive fibrosing interstitial lung disease (PF-ILD) are linked to mortality.
  • Lung function trajectory after meeting PF-ILD criteria is largely unknown.
  • Predicting forced vital capacity (FVC) change is vital for clinical trial design.

Purpose of the Study:

  • To evaluate how individual PF-ILD criteria predict subsequent FVC change.
  • To determine if PF-ILD criteria can be refined for better clinical trial design.
  • To assess the influence of ILD subtype on FVC trajectory after PF-ILD diagnosis.

Main Methods:

  • Retrospective, multicentre longitudinal cohort analysis of 1227 patients.
  • Included patients with fibrotic connective tissue disease-associated ILD (CTD-ILD), chronic hypersensitivity pneumonitis, and idiopathic interstitial pneumonia.
  • Joint modelling used to estimate 1-year FVC change after meeting PF-ILD criteria.

Main Results:

  • Six of nine PF-ILD criteria were associated with differential 1-year FVC change.
  • Radiological progression of fibrosis showed the largest FVC decline.
  • FVC change varied significantly by ILD subtype, with CTD-ILD showing less change.

Conclusions:

  • Near-term FVC change after meeting PF-ILD criteria is heterogeneous.
  • ILD subtype significantly influences FVC trajectory and should be considered when applying PF-ILD criteria.
  • Findings can inform future clinical trial design for interstitial lung diseases.