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Small airway involvement in cystic fibrosis lung disease: routine spirometry as an early and sensitive marker

E Marije Bakker1, Gerard J J M Borsboom, Els C van der Wiel-Kooij

  • 1Department of Pediatric Pulmonology and Allergology, Erasmus MC-Sophia Children's Hospital, Rotterdam, The Netherlands.

Pediatric Pulmonology
|February 13, 2013
PubMed

Insights

Forced expiratory flow at 75% (FEF75) is a more sensitive marker for early cystic fibrosis (CF) lung disease than FEV1 or FVC. This finding supports using FEF75 in clinical trials for CF patients.

Area of Science:

  • Pulmonary Medicine
  • Pediatric Respiratory Health
  • Clinical Diagnostics

Background:

  • Early detection of obstructive lung disease in young cystic fibrosis (CF) patients is crucial.
  • Standard spirometry measures like FEV1 can be normal in early-stage CF, necessitating more sensitive indicators.

Purpose of the Study:

  • To assess the age-related progression of spirometry parameters in CF patients compared to healthy children.
  • To identify the most sensitive spirometry marker for early detection of lung disease in CF.

Main Methods:

  • Retrospective analysis of longitudinal spirometry data from CF patients and healthy cohorts (ages 6-20).
  • Quantile regression used to determine 10th, 50th, and 90th percentiles for FVC, FEV1, and FEF75.
  • Sample size calculations performed using these parameters as potential clinical trial endpoints.

Main Results:

  • CF patients exhibited significantly lower FVC, FEV1, and FEF75 compared to healthy controls.
  • Abnormalities in FEF75 were detected at younger ages and were more pronounced than FEV1 or FVC abnormalities throughout childhood.
  • FEF75 demonstrated greater sensitivity for detecting lung disease in CF patients.

Conclusions:

  • FEF75 is a more sensitive marker for early cystic fibrosis lung disease than FEV1 and FVC.
  • The earlier and more substantial decline in FEF75 supports its use as a primary endpoint in clinical trials for CF.
  • Utilizing FEF75 may reduce the sample size needed to detect treatment effects in CF trials.
Abstract

Keywords:
cystic fibrosispediatricssmall airway diseasespirometry

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