From the Guidelines
Cystic bronchiectasis is primarily caused by conditions that lead to permanent bronchial wall damage, including childhood respiratory infections, congenital disorders, and underlying diseases that impair host immunity.
Causes of Cystic Bronchiectasis
- Childhood respiratory infections: Such as bacterial pneumonia, pertussis, or measles, which can cause permanent bronchial wall damage 1.
- Congenital disorders: Including cystic fibrosis (CF), primary ciliary dyskinesia, and alpha-1 antitrypsin deficiency, which can increase the risk of developing bronchiectasis 1.
- Underlying diseases that impair host immunity: Such as HIV infection, common variable immunodeficiency, and allergic bronchopulmonary aspergillosis (ABPA), which can predispose individuals to recurrent infections and bronchiectasis 1.
- Other conditions: Including rheumatoid arthritis, inflammatory bowel disease, and gastroesophageal reflux disease (GERD), which may also contribute to the development of bronchiectasis, although the relationship between these conditions and bronchiectasis is not always clear 1.
Pathophysiology
The destruction of the elastic and muscular components of bronchial walls, combined with the contractile force of surrounding lung tissue, can lead to the expansion of the involved airways and the formation of cystic bronchiectasis 1.
Diagnosis and Management
Identifying the underlying cause of bronchiectasis is crucial, as it can inform treatment decisions and improve patient outcomes 1. A systematic approach to diagnosis, including imaging studies, laboratory tests, and clinical evaluation, can help identify the underlying cause of bronchiectasis and guide management decisions.
From the Research
Causes of Cystic Bronchiectasis
The causes of cystic bronchiectasis are multifactorial and can be attributed to various factors, including:
- Genetic predisposition, with different molecules implicated in the onset and development of idiopathic bronchiectasis 2
- Infections, such as bacterial or fungal infections, which can lead to inflammation and damage to the airways 3, 4
- Post-infectious effects, which can cause bronchiectasis as a complication of previous lower respiratory infection and/or inflammation 5, 6
- Underlying lung diseases, such as chronic obstructive pulmonary disease (COPD) or severe asthma, which can increase the risk of developing bronchiectasis 3, 6
- Allergic bronchopulmonary aspergillosis (ABPA), which can complicate asthma and lead to bronchiectasis 3, 4
- Primary ciliary dyskinesia, primary immunodeficiencies, and certain systemic diseases, such as inflammatory bowel disease and rheumatoid arthritis, which can also contribute to the development of bronchiectasis 5, 6
- Foreign body inhalation, which can cause bronchiectasis as a result of airway obstruction and inflammation 6
Specific Causes
Some specific causes of cystic bronchiectasis include:
- Cystic fibrosis, which is a common cause of bronchiectasis, although it is excluded from some reviews 2, 4
- Aspergillus-associated syndromes, such as aspergilloma and fungal bronchitis, which can develop in areas of lung damage seen in patients with bronchiectasis 3, 4
- Invasive aspergillosis, which can occur in patients with underlying lung diseases, including bronchiectasis 3
- Traction bronchiectasis, which can be caused by prior tuberculosis, sarcoidosis, and silicosis with progressive massive fibrosis 5