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Updated: Dec 24, 2025
Long Term Chronic Pseudomonas aeruginosa Airway Infection in Mice
Published on: March 17, 2014
Exacerbations and Pseudomonas aeruginosa colonization are associated with altered lung structure and function in
G Piatti1, M M De Santi2, A Farolfi3
1Department of Pathophysiology and Transplantation, University of Milan and Unit of Bronchopneumology, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Via Francesco Sforza 35 -, 20122, Milan, Italy. gioia.piatti@unimi.it.
Background:
Recurrent bacterial infections of the respiratory tract are one of the major clinical features of the primary ciliary dyskinesia (PCD), a rare genetic disease due to malfunctioning of motile cilia. Chronic infections and persistent inflammation of the respiratory system result in progressive lung disease. Aim of the study was to highlight the main factors associated with clinical, functional and anatomical deterioration in PCD patients.
Methods:
We retrospectively analyzed data from 58 patients with PCD, 37 adults and 21 children. The demographic and clinical data, forced expiratory volume at 1 s (FEV1) and forced vital capacity (FVC), sputum microbiology and imaging results (chest CT scores-modified Bhalla) were recorded. Patients were stratified according to the number of exacerbations (< 2/year vs ≥ 2/year) and chronic Pseudomonas aeruginosa (PA) colonization. The possible correlations between lung function and chest CT scores were assessed; we also evaluated the correlation between these parameters and the severity scores for bronchiectasis (BSI, FACED and e-FACED).
Results:
Chest CT scores showed a significant correlation with FEV1 (p = 0.0002), age (p < 0.0001), BMI (p = 0.0002) and number of lung lobes involved (p < 0.0001). PA colonization had an overall prevalence of 32.6%: no significant difference in FEV1 between PA colonized and non-colonized patients was found (p = 0.70), while chest CT score was significantly worse in chronic PA colonized patients (p = 0.009). Patients with a high number of exacerbation (≥ 2/year) were older (p = 0.01), had lower FEV1 (p = 0.03), greater number of lobes involved (p < 0.001) and worse CT score than patients with low number of exacerbations (p = 0.001); they also had higher prevalence of PA chronic bronchial infection (33.3% versus 13.6%, p = 0.10). Multivariable linear regression analyses adjusted for gender, age and BMI showed positive associations between PA colonization and number of exacerbations with severity of disease (number of lobes involved, CT score, BSI, FACED, and e-FACED).
Conclusions:
In our PCD population the number of exacerbations (≥ 2/year) and PA colonization were the two most relevant factors associated with severity of disease.
Insights
Recurrent bacterial infections and Pseudomonas aeruginosa colonization significantly worsen lung disease severity in primary ciliary dyskinesia (PCD). Frequent exacerbations (≥2/year) are key indicators of disease progression in PCD patients.
Area of Science:
- Genetics and Respiratory Medicine
- Rare Diseases Research
- Clinical Investigations
Background:
- Primary ciliary dyskinesia (PCD) is a rare genetic disorder characterized by impaired motile cilia function.
- Recurrent respiratory tract infections and chronic inflammation lead to progressive lung disease in PCD patients.
- Identifying factors associated with disease deterioration is crucial for managing PCD.
Purpose of the Study:
- To identify key factors contributing to clinical, functional, and anatomical decline in patients with primary ciliary dyskinesia.
- To investigate the correlation between respiratory infections, lung function, and disease severity in PCD.
Main Methods:
- Retrospective analysis of data from 58 PCD patients (37 adults, 21 children).
- Collected data included demographics, lung function (FEV1, FVC), sputum microbiology, and chest CT scores.
- Patients were stratified by exacerbation frequency (≥2/year vs. <2/year) and chronic Pseudomonas aeruginosa (PA) colonization.
Main Results:
- Chest CT scores correlated significantly with FEV1, age, BMI, and the number of lobes affected.
- Chronic PA colonization was associated with significantly worse chest CT scores.
- Patients with frequent exacerbations (≥2/year) exhibited older age, lower FEV1, more affected lobes, and worse CT scores.
- Multivariable analysis confirmed PA colonization and exacerbation frequency as significant predictors of disease severity.
Conclusions:
- In this PCD cohort, frequent exacerbations (≥2/year) and PA colonization are the most significant factors associated with disease severity.
- These findings highlight the importance of managing respiratory infections and monitoring for PA colonization in PCD patients.
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