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Pulmonary Exacerbations and Treatment in PCD: A Narrative Review
Pinelopi Anagnostopoulou1, Stephanie Thee2,3,4,5, Panayiotis Kouis1
1Medical School, University of Cyprus, Nicosia, Cyprus.
Pediatric Pulmonology
|July 1, 2026
Summary
Pulmonary exacerbations (PEx) in primary ciliary dyskinesia (PCD) cause chronic lung disease. Understanding PEx triggers and improving treatments are crucial for managing this rare genetic disorder.
Area of Science:
- Pulmonology
- Genetics
- Rare Diseases
Background:
- Primary ciliary dyskinesia (PCD) is a rare genetic disorder leading to chronic lung disease.
- Patients with PCD frequently experience pulmonary exacerbations (PEx), characterized by symptom worsening and lung function decline.
Purpose of the Study:
- To review current knowledge on PEx in PCD.
- To identify understudied areas for future research.
Main Methods:
- Literature review of PCD-specific PEx definitions and frequency.
- Analysis of studies on PEx pathophysiology, including microorganisms and inflammatory markers.
- Identification of risk factors and prognostic indicators for PEx.
Main Results:
- PEx definitions vary in the literature.
- Common PEx triggers include Pseudomonas aeruginosa infection.
- Female sex and age are identified risk factors; lung clearance index is a prognostic factor.
Conclusions:
- Further research is essential to elucidate PEx mechanisms in PCD.
- Improved understanding is needed for effective treatment and prevention strategies.
- Long-term consequences of PEx in PCD require further investigation.
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