Management of Cystic Bronchiectasis

Diagnostic Workup

The European Respiratory Society recommends a minimum bundle of aetiological tests, including differential blood count, serum immunoglobulins, testing for allergic bronchopulmonary aspergillosis, and sputum culture and sensitivity, when cystic bronchiectasis is newly diagnosed 1, 4
Sputum culture and sensitivity should be performed for bacterial monitoring, with mycobacterial culture in selected cases where non-tuberculous mycobacteria are suspected 1, 4

The European Respiratory Society recommends airway clearance techniques, such as active cycle of breathing, autogenic drainage, or devices like Flutter or Acapella, for 10-30 minutes once or twice daily, for all patients with chronic productive cough or difficulty expectorating sputum 1, 2, 3
Pulmonary rehabilitation is strongly recommended for all patients with impaired exercise capacity, consisting of 6-8 weeks of supervised exercise training, which improves exercise capacity and reduces exacerbation frequency 2, 3, 4

The European Respiratory Society recommends using bronchodilators before physiotherapy sessions, inhaled mucoactive drugs, and inhaled antibiotics to increase tolerability and optimize pulmonary deposition 8, 3
Long-term mucoactive treatment is suggested for patients with difficulty expectorating sputum and poor quality of life where standard airway clearance techniques have failed 8, 3

The European Respiratory Society recommends treating all acute exacerbations with 14 days of antibiotics, with selection based on previous sputum culture results 1, 4, 5, 6
Common pathogens and first-line treatments include Streptococcus pneumoniae (amoxicillin), Haemophilus influenzae (amoxicillin), and Pseudomonas aeruginosa (ciprofloxacin) 5, 6

The European Respiratory Society recommends considering long-term antibiotics only for patients with ≥3 exacerbations per year, with first-line treatment being long-term inhaled antibiotics for chronic Pseudomonas aeruginosa infection 1, 8
Macrolides (azithromycin or erythromycin) are suggested for patients with ≥3 exacerbations per year without Pseudomonas aeruginosa infection 1, 8

The European Respiratory Society recommends against offering inhaled corticosteroids to adults with bronchiectasis unless comorbid asthma or COPD is present, and against offering statins for treatment of bronchiectasis 1, 8, 6

The European Respiratory Society recommends surgery only for highly selected patients with localized disease and high exacerbation frequency despite optimization of all other management aspects, with a pooled mortality from surgery of 1.4% and post-operative morbidity of 16.2% 1, 8, 3, 4

The European Respiratory Society recommends annual assessments, with more frequent monitoring in severe disease, and performing pulse oximetry to screen for respiratory failure, and monitoring sputum culture and sensitivity regularly 5