What is the recommended diagnostic and management strategy for bronchiectasis, including evaluation for underlying causes, airway clearance techniques, infection control, and comorbidity management?

Bronchiectasis: Diagnostic and Management Strategy

Diagnosis

High-resolution CT (HRCT) scanning is the diagnostic procedure of choice for bronchiectasis, with sensitivity and specificity exceeding 90%. 1, 2 Do not rely on physical examination findings such as crackles or rhonchi, as these are nonspecific and can be normal even in confirmed bronchiectasis. 2

Clinical Features That Should Trigger Investigation

• Chronic productive cough with mucopurulent or purulent sputum is the cardinal feature. 1, 2
• Cough persisting longer than 8 weeks with sputum production. 1, 2
• Recurrent chest infections, particularly in at-risk populations (HIV, transplant recipients, immunosuppressive therapy, connective tissue disease, inflammatory bowel disease, chronic rhinosinusitis). 1

Initial Diagnostic Workup

Obtain the following minimum bundle of tests to identify treatable underlying causes: 2, 3, 4

• Complete blood count with differential 5
• Serum immunoglobulins (IgG, IgA, IgE, IgM) 2, 3, 5
• Testing for allergic bronchopulmonary aspergillosis (ABPA) 2, 3
• Sputum culture for bacteria, mycobacteria, and fungi 3, 5
• Prebronchodilator and postbronchodilator spirometry 5
• Serum protein electrophoresis if immunoglobulins are elevated. 1
• Consider HIV serology based on risk factors. 1
• Consider bronchoscopy for localized disease to rule out endobronchial lesion or foreign body. 1

Management Strategy

Core Non-Pharmacological Interventions

Airway clearance techniques are the cornerstone of therapy and must be taught by a respiratory physiotherapist. 1, 2, 6 All patients with chronic productive cough should perform these techniques once or twice daily for 10-30 minutes. 2, 6, 3

Recommended airway clearance techniques: 1, 2

• Active cycle of breathing techniques
• Oscillating positive expiratory pressure devices
• Gravity-assisted positioning (where not contraindicated) to enhance effectiveness
• Forced expiration technique (huff) should be incorporated

Pulmonary rehabilitation is strongly recommended for patients with impaired exercise capacity, consisting of 6-8 weeks of supervised exercise training to improve exercise capacity, reduce cough symptoms, and enhance quality of life. 6

Pharmacological Management

Bronchodilators

• Use bronchodilators if significant breathlessness is present, particularly with chronic obstructive airflow limitation. 6, 3, 5
• Ensure proper inhaler technique training and appropriate device selection. 6

Mucoactive Agents

• Consider nebulized sterile water or normal saline to facilitate airway clearance. 2, 6
• Do NOT use recombinant human DNase (dornase alfa) in non-CF bronchiectasis, as it may worsen outcomes. 2, 6

Corticosteroids

• Do NOT routinely offer inhaled corticosteroids unless other indications exist (ABPA, asthma, COPD, or inflammatory bowel disease). 2, 6
• Do NOT use long-term oral corticosteroids without specific indications. 6

Infection Control Strategy

Pathogen Identification

Obtain sputum culture prior to starting antibiotics to identify pathogens, particularly Haemophilus influenzae and Pseudomonas aeruginosa. 2, 6 P. aeruginosa infection is associated with three-fold increased mortality risk, seven-fold increased hospitalization risk, and one additional exacerbation per year. 1, 6

Exacerbation Management

Treat all exacerbations with 14 days of oral or intravenous antibiotics based on previous sputum culture results. 1, 2, 6, 3, 5

• Empirical antibiotics can be started while awaiting sputum microbiology, then modified based on sensitivity results if no clinical improvement. 2
• First-line treatments: amoxicillin for Streptococcus pneumoniae and Haemophilus influenzae; ciprofloxacin for Pseudomonas aeruginosa. 6
• Intravenous antibiotics are indicated for severely unwell patients, resistant organisms, or failed oral therapy. 2

Long-Term Antibiotic Therapy

Consider long-term antibiotic therapy (azithromycin or inhaled antibiotics such as colistin or gentamicin) ONLY for patients with ≥3 exacerbations per year, after optimizing airway clearance techniques and treating underlying causes. 6, 3, 5

• For mild disease without frequent exacerbations (<3 per year), long-term antibiotics are NOT indicated. 6
• Regular monitoring of sputum pathogens and drug toxicity is required, especially with macrolides and inhaled aminoglycosides. 3

Immunizations

Annual influenza vaccination is mandatory for all bronchiectasis patients. 6, 3, 5

Pneumococcal vaccination is recommended for all bronchiectasis patients. 6, 3, 5

Monitoring and Follow-Up

• Use the bronchiectasis severity index to guide management intensity. 1, 2
• Assess patients annually for mild disease, more frequently in severe disease. 2, 6
• Patients admitted with exacerbations should be seen daily by a respiratory physiotherapist until airway clearance is optimized. 1, 2
• Review airway clearance technique within 3 months of initial assessment. 2
• Pulse oximetry should be used to screen for respiratory failure. 6

Surgical Considerations

Surgery is NOT recommended for mild disease; reserve only for localized disease with high exacerbation frequency despite optimization of all medical therapy. 1, 6, 3

Lung transplant may be considered for patients with severely impaired pulmonary function or frequent exacerbations. 5

Critical Pitfalls to Avoid

• Do NOT extrapolate treatments from cystic fibrosis bronchiectasis, as treatment responses differ significantly. 1, 6
• Do NOT use inhaled DNase in non-CF bronchiectasis. 2, 6
• Do NOT routinely use inhaled corticosteroids without comorbid asthma or COPD. 2, 6
• Do NOT neglect to investigate for underlying causes, as up to 62% of cases have identifiable treatable etiologies. 5, 4