Permanent bronchial dilatation from chronic infection and inflammation
Expansion
Causes, using A SICK AIRWAY:
- A - Allergic bronchopulmonary aspergillosis
- S - Sequestration and sarcoidosis
- I - Infection: childhood pertussis or measles, tuberculosis, severe pneumonia
- C - Cystic fibrosis
- K - Kartagener’s syndrome (primary ciliary dyskinesia, with situs inversus and infertility)
- A - Aspiration, and alpha-1 antitrypsin deficiency
- I - Immunodeficiency, especially hypogammaglobulinaemia
- R - Rheumatoid arthritis and connective tissue disease
- W - Williams-Campbell syndrome, a cartilage deficiency
- A - Airway obstruction: tumour or foreign body
- Y - Yellow nail syndrome
Features: chronic cough with large volumes of purulent sputum, haemoptysis, recurrent infection, coarse crackles that change with coughing, wheeze and clubbing.
Diagnosis is by high resolution CT, showing the signet ring sign, where the dilated bronchus is larger than its accompanying artery, together with lack of tapering and visible bronchi within 1 cm of the pleura.
Management: airway clearance and physiotherapy are the foundation, plus prompt treatment of exacerbations, vaccination, treating the underlying cause, and long term azithromycin in frequent exacerbators. Chronic Pseudomonas colonisation marks a worse prognosis and requires targeted treatment.