Diagnosis and early life risk factors for bronchiectasis in cystic fibrosis: a review

ABSTRACT

Introduction: Lung disease in cystic fibrosis begins in early life with neutrophil-dominated inflammation and infection, is progressive and results in structural lung damage characterised by bronchial dilation and bronchiectasis. Preventative strategies must be employed in early life but require a better understanding of how bronchiectasis develops.

Areas covered: In this review we have addressed the diagnosis and early life risk factors for bronchiectasis in young children with cystic fibrosis. A systematic review was not performed and the literature reviewed was known to the authors.

Expert commentary: Bronchiectasis represents a process of progressive dilatation and damage of airway walls and is traditionally considered to be irreversible. Diagnosis is primarily by detecting a bronchial:arterial ratio of >1 on chest CT scan. Lung volume has a greater influence on airway diameter than on arterial making control of lung volume during scanning critical. Early life risk factors for the onset and progression bronchiectasis include: severe cystic fibrosis genotype; neutrophilic inflammation with free neutrophil elastase activity in the lung; and pulmonary infection. Bronchiectasis develops in the majority of children before they reach school age despite the best current therapy. To prevent bronchiectasis novel therapies are going to have to be given to infants.

KEYWORDS:

• Paediatrics
• radiology
• chest computer tomography
• CFTR
• CFTR modulator
• CFTR potentiator
• neutrophilic inflammation

Declaration of interest

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.