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Published on: January 6, 2015
Change in IgG and evolution of lung function in children with cystic fibrosis
M Proesmans1, C Els, F Vermeulen
1Dept. Pediatrics, Pediatric Pulmonology University Hospital of Leuven, Belgium. Marijke.proesmans@uz.kuleuven.be
Insights
In cystic fibrosis (CF) patients, rising immunoglobulin G (IgG) levels correlate with worsening lung function (FEV1). This study monitored IgG in pediatric CF patients over four years, finding increased IgG levels linked to declining lung capacity.
Area of Science:
- Pediatric Pulmonology
- Immunology
- Chronic Disease Management
Background:
- Previous studies linked high immunoglobulin G (IgG) levels in cystic fibrosis (CF) patients with severe lung disease and poor prognosis.
- Current standards of care for CF aim to mitigate disease progression, necessitating updated outcome parameters.
Purpose of the Study:
- To investigate immunoglobulin G (IgG) levels as a potential outcome measure for lung disease severity in pediatric cystic fibrosis (CF) patients.
- To assess the relationship between IgG levels and lung function (FEV1) in a cohort of children with CF.
Main Methods:
- A 4-year longitudinal study of 73 pediatric CF patients (aged 5-15 years at baseline) treated under current standards of care.
- Data collected included spirometry (FEV1), height, weight, sputum cultures, and total IgG levels.
- Statistical analysis focused on IgG z-scores and their correlation with FEV1% predicted, both cross-sectionally and longitudinally.
Main Results:
- The prevalence of patients with IgG z-scores >2 SD increased from 16% in 2004 to 25% in 2008.
- IgG z-scores showed a significant inverse correlation with FEV1% predicted (r=-0.323 in 2004; p<.001).
- Longitudinal analysis revealed that changes in IgG z-score were inversely correlated with changes in FEV1% predicted (r=-0.498; p<.001).
Conclusions:
- Even in pediatric CF patients receiving current standards of care, IgG z-scores tend to increase with age.
- Elevated IgG levels are associated with a decline in lung function (FEV1) in pediatric CF patients.
- IgG z-score may serve as a valuable indicator of lung disease progression in pediatric CF.
Abstract:
Reports from the seventies and eighties have shown that cystic fibrosis (CF) patients with severe lung disease have high levels of IgG and that this is associated with worse prognosis. We decided to explore IgG level as a possible outcome parameter for lung disease severity in a cohort of pediatric CF patients treated according to current standards of care. Seventy three CF children older than 5 years (and max 15 years old at the initial evaluation) attending the same CF center were followed during a period of 4 years. Data collection included spirometry, height, weight, sputum cultures and total IgG. Median age at the start was 10 years. IgG z scores<2 SD were seen in 2.7% of patients in 2004 and 2008. Twelve patients (16%) had an IgG>2 SD in 2004 and this number increased to 18 (25%) in 2008. IgG z-scores were inversely correlated with FEV(1)% predicted (r=-0.323 in 2004; p<.001). In longitudinal evaluation, changes in IgG z-score correlate inversely with changes in FEV(1)% predicted (r=-0.498; p<.001). We can conclude that even for CF patients treated according to current standards IgG z-score increases with age and is correlated with a decline in FEV(1).
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