Bronchiectasis
Basics
- Irreversible bronchial dilation with chronic productive cough and recurrent exacerbations
- Typical imaging: bronchial wall thickening, dilatation, "tree-in-bud" nodularity
Epidemiology
- Prevalence in US: 701 per 100,000; higher in women and older adults
Etiology and Pathophysiology
- Arises from inherited or acquired diseases or idiopathic
- Vicious cycle: initial injury β airway inflammation β impaired clearance β infection β progression
- NETs and neutrophil elastase correlate with disease severity; potential therapeutic targets
Prevention
- Vaccinations: pertussis, measles, Hib, influenza, pneumococcus
- Early treatment of respiratory and inflammatory diseases
- Smoking cessation
- Genetic counseling for inheritable predispositions
Associated Conditions
- Acquired: pneumonia, GERD, asthma, COPD, rheumatoid arthritis, TB, ABPA, rhinosinusitis, airway obstruction
- Inherited: cystic fibrosis, primary ciliary dyskinesia, tracheobronchomalacia, Ξ±1-antitrypsin deficiency
Diagnosis
History
- Chronic cough with sputum most days
- Dyspnea, fatigue, rhinosinusitis
- Recurrent exacerbations with increased cough, sputum volume/purulence, breathlessness, fatigue, hemoptysis
Physical Exam
- Basal coarse rales, wheezing
- Evaluate for alternative diagnoses
Differential Diagnosis
- COPD, asthma, chronic rhinosinusitis, lung cancer, GERD
Diagnostic Tests
- Pulmonary function tests: often normal or obstructive
- Chest X-ray: limited sensitivity; may show atelectasis, airway dilation
- High-resolution CT chest (slice β€1 mm): diagnostic modality of choice
- Labs: CBC, immunoglobulins, ABPA testing
- Sputum cultures for bacteria, mycobacteria, fungi
- Additional testing for TB, HIV, primary ciliary dyskinesia, GERD, immunodeficiency
CT Findings Diagnostic of Bronchiectasis
- Bronchoarterial ratio >1
- Lack of airway tapering
- Visible airways near pleural surface or mediastinum
- Mucus plugging, bronchial wall thickening, "tree-in-bud" pattern, mosaic perfusion
Treatment
Stepwise Management
- Treat underlying cause
- Airway clearance techniques; pulmonary rehabilitation
- Vaccinations: pneumococcal and annual influenza
- Manage exacerbations promptly with empiric antibiotics, guided by sputum cultures
- Consider mucolytics for sputum clearance difficulty
- Long-term antibiotics for frequent exacerbations (>3/year)
- IV antibiotics for refractory cases
Antibiotics
- First line:
- Amoxicillin (S. pneumoniae, H. influenzae Ξ²-lactamase negative)
- Amoxicillin/clavulanic acid (Ξ²-lactamase positive H. influenzae, Moraxella catarrhalis)
- Flucloxacillin (S. aureus)
-
Ciprofloxacin (P. aeruginosa)
-
Duration: 14 days for exacerbations
-
Eradication of new Pseudomonas: oral fluoroquinolone 2 weeks + inhaled antibiotics for 3 months
-
Second line: doxycycline, clarithromycin
Referral Indications
- β₯3 exacerbations per year
- Positive sputum for P. aeruginosa, MRSA, NTM, or ABPA
- Long-term antibiotics use
- Rapid deterioration or lung transplantation consideration
Additional Therapies
- Mucoactive agents: dry powder mannitol, nebulized hypertonic saline
- Long-acting bronchodilators for breathlessness
- Pulmonary rehabilitation
- Nutrition, exercise, psychological support
Surgery
- Consider for localized disease with frequent exacerbations refractory to medical therapy
- Lung transplantation for progressive deterioration
- Bronchial aspiration or wash for refractory sputum clearance
Inpatient Care
- Admission for oxygen requirement, IV meds, or outpatient failure
Follow-Up
- Clinic visits every 3-6 months
- Monitor sputum cultures every 6-12 months
- Pulmonary function and imaging as indicated
- Reassess airway clearance and therapies regularly
Patient Education
Prognosis
- Use validated severity scores (Bronchiectasis Severity Index, FACED score)
- Poor prognosis factors: Pseudomonas infection, low BMI, advanced age
Complications
- Hemoptysis
- Pneumothorax
- Sepsis, lung abscess
- Pulmonary hypertension, cor pulmonale