Table 1:

Causes of bronchiectasis (4) (9)

CauseMean incidenceSupporting diagnostic featuresDiagnostic investigations
Cystic fibrosis0.6%–2.7%Younger age (< 45 yr); history of malabsorption; history of pancreatitis; history of Pseudomonas aeruginosa infection, Staphylococcus aureus infection, nontuberculous mycobacterial infection; history of male infertilitySweat chloride assessment; CFTR genetic analysis as per guidelines (specialist centre referral)
Alpha1-antitrypsin deficiency0.6%–11.3%Evidence of emphysema; obstructive pattern on spirometry; panniculitisSerum alpha1-antitrypsin level; phenotyping in those with low serum levels
Primary ciliary dyskinesia2.0%–10.3%History of chronic upper respiratory tract problems, otitis media, male infertility; abnormal ciliary beat pattern ± frequency on nasal brushingsMeasurement of nasal nitric oxide levels; ciliated epithelial biopsy (specialist centre referral)
Allergic bronchopulmonary aspergillosis0.9%–7.8%History of asthma; peripheral blood eosinophils > 500 cells/μL; positive Aspergillus fumigatus IgG or positive precipitins; sputum culture of A. fumigatus; fleeting infiltrates on chest radiograph or CT chest; proximal bronchiectasis on CT chest scanTotal IgE > 500 IU/mL; positive A. fumigatus–specific IgE or immediate reaction on skin-prick testing
Autoimmune/connective tissue diseases (typically rheumatoid arthritis, SLE)1.8%–31.1%History or clinical signs of connective tissue disease ± vasculitisRheumatoid factor; anti-CCP; other investigations pertinent to suspected diagnosis from clinical review
Inflammatory bowel diseases1.0%–3.0%History or clinical signs of ulcerative colitis or Crohn diseaseSpecialist gastrointestinal review; positive pathological features on colonoscopy
Congenital malformations0.2%–0.6%Williams–Campbell syndrome (bronchomalacia); Mounier-Kuhn syndrome (tracheobronchomegaly) and lung sequestrationTypically diagnosed on chest CT
Aspiration0.2%–11.3%History of reflux; history of aspirationVarious modalities available: video fluoroscopic swallow study; upper gastrointestinal endoscopy; ambulatory esophageal manometry; pH studies; flexible endoscopic evaluation of swallow
Humoral immunodeficiency1.1%–16.0%History of recurrent infectionsSerum immunoglobulins levels (IgG, IgA and IgM); specific antibody responses to pneuomococcal, Haemophilus influenzae B and tetanus antigens
Postinfectious*29.0%–42.0%History or radiologic evidence of previous infection (e.g., frequently, pneumonia, Bordetella pertussis, Mycobacterium tuberculosis, nontuberculous mycobacteria)
Idiopathic*26.0%–53.0%Other causes excludedOther causes excluded
  • Note: CCP = cyclic citrullinated peptide; CFTR = cystic fibrosis transmembrane conductance regulator; CT = computed tomography, Ig = immunoglobulin, SLE = systemic lupus erythematosus.

  • * This review focuses predominantly on the management of these 2 causes of bronchiectasis.