Table 1

Common aetiologies with their clinical features, investigations and specific therapies

Cause of bronchiectasisClinical featuresInvestigationsSpecific treatment
NTM Pulmonary diseaseElderly females, middle lobe nodular bronchiectatic or cavitary disease
Weight loss
Sputum culture
HRCT appearances may be suggestive
Combination antibiotic treatment for at least 12 months after sputum culture conversion
ABPAWheezing, mucus production, steroid responsiveness. Central bronchiectasis
Staphylococcus aureus infection
Total and specific IgE, eosinophil counts
HRCT appearances may be suggestive
Oral corticosteroids
Antifungal medication
Primary immunodeficiencyRecurrent infections, extrapulmonary infectionsSerum immunoglobulins and functional antibodies, e.g. to pneumococcusImmunoglobulin replacement
Prophylactic antibiotics, e.g. macrolides
Secondary immunodeficiencyRecurrent infections, extrapulmonary infections, symptoms of the underlying disorder (e.g. haematological malignancy)Serum immunoglobulins, more detailed immunological investigationsDiscontinue immunosuppressive medications
Treat underlying disorder
Immunoglobulin replacement
Prophylactic antibiotics, e.g. macrolides
Connective tissue diseaseProgressive disease with frequent exacerbationsClinical history supported by autoantibody measurementsAirway clearance and early introduction of anti-inflammatory treatment with macrolides if frequent exacerbations
Inflammatory bowel diseaseSterile bronchorrhoeaClinical historyInhaled corticosteroids
Alpha-1 antitrypsin deficiencyBasal emphysema
Airflow obstruction
Alpha-1 antitrypsin serum level and phenotypingSmoking cessation
Treat airflow obstruction with bronchodilators
Supplementation in some countries
PCDChildhood respiratory distress or respiratory symptoms
Upper airway symptoms
Middle ear infections
Cardiac involvement/dextrocardia
Early infection with Pseudomonas aeruginosa
Nasal NO
High-speed video microscopy
Electron microscopy
Airway clearance
Genetic counselling
Assessment of fertility and cardiac involvement
Monitoring and early treatment of P. aeruginosa
Cystic fibrosisChildhood respiratory symptoms
Extrapulmonary features
Upper airway symptoms
Infection with P. aeruginosa or S. aureus
Sweat test
Airway clearance
Multidisciplinary assessment and management within specialist centres
Specific therapies not indicated in bronchiectasis, e.g. DNAse
CFTR modulators
ObstructiveSingle lobe involvementBronchoscopyRemoval of obstructive lesion if possible
Congenital, e.g. tracheobronchomegaly, Williams-­Campbell syndromeTypical radiological appearances
Family history
HRCT appearances may be suggestiveNone
Yellow nail syndromeYellow discolouration of fingernails and toenails
Pleural effusions
Clinical featuresTreatment of complications, e.g. drainage of pleural effusions

NTM: nontuberculous mycobacteria; HRCT: high-resolution computed tomography; NO: nitric oxide; CFTR: cystic fibrosis transmembrane conductance regulator.