Bronchiectasis
Permanent abnormal dilation of bronchi with chronic infection and inflammation.
Also known as: bronchiectasis, chronic suppurative lung disease, tram-tracking
Overview
Irreversible dilation and thickening of medium-sized bronchi resulting from chronic inflammation and recurrent infection, producing chronic productive cough, recurrent exacerbations, and progressive lung damage.
Epidemiology
Underdiagnosed but increasingly recognized — prevalence ~50-500 per 100,000 in the US. More common in older women and those with prior respiratory infection or underlying lung disease. Cystic fibrosis is a major cause in younger patients.
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Risk factors
- Post-infectious: severe pneumonia (pertussis, measles, adenovirus, TB, NTM)
- Cystic fibrosis (CFTR mutations)
- Primary ciliary dyskinesia (Kartagener: situs inversus + bronchiectasis + sinusitis)
- Immunodeficiency (CVID, hypogammaglobulinemia, HIV)
- Allergic bronchopulmonary aspergillosis (ABPA)
- Chronic aspiration, GERD
- Connective tissue disease: rheumatoid arthritis, Sjögren
- Inflammatory bowel disease, alpha-1 antitrypsin deficiency
- Mounier-Kuhn (tracheobronchomegaly), Williams-Campbell (cartilage deficiency)
Pathophysiology
Vicious cycle of impaired mucociliary clearance, chronic bacterial colonization (Pseudomonas, H. influenzae, S. aureus, Moraxella, NTM), neutrophilic inflammation, and bronchial wall destruction. Permanent bronchial dilation, fibrosis, and loss of elastic recoil follow.
Clinical presentation
Symptoms
- Chronic productive cough with copious mucopurulent sputum (often daily, worse in morning)
- Recurrent respiratory infections / exacerbations
- Dyspnea, wheezing, fatigue
- Hemoptysis (variable; ranges from blood-streaked sputum to massive)
- Pleuritic chest pain during exacerbations
Signs / physical exam
- Coarse crackles (often persistent), wheezes, rhonchi
- Clubbing in advanced disease
- Cyanosis, signs of cor pulmonale if severe
Differential diagnosis
- COPD — Smoking history, obstructive PFTs, hyperinflation; less mucopurulent sputum; can coexist
- Asthma — Reversible airflow obstruction, atopic features; can coexist (especially with ABPA)
- Cystic fibrosis — Younger onset, pancreatic insufficiency, infertility, elevated sweat chloride
- Tuberculosis — Apical cavitary disease; positive AFB/IGRA; can cause post-infectious bronchiectasis
- Non-tuberculous mycobacterial disease — 'Lady Windermere' — older women with right middle lobe/lingula bronchiectasis and nodules; M. avium complex
- Chronic eosinophilic pneumonia / EGPA — Peripheral eosinophilia, asthma overlap
- Recurrent aspiration — Posterior or dependent segments; underlying neurologic or esophageal disorder
Diagnostic workup
Labs
- Sputum culture (bacterial, mycobacterial, fungal) — Pseudomonas isolation predicts severity
- CBC, immunoglobulins (IgG, IgA, IgM, IgE), HIV
- Sweat chloride test and/or CFTR gene panel if CF suspected
- Aspergillus-specific IgE and total IgE for ABPA
- Alpha-1 antitrypsin level
- Autoimmune panel (RF, ANA) if CTD suspected
Imaging
- High-resolution CT chest is the diagnostic gold standard — findings: bronchial dilation (signet-ring sign, internal lumen larger than adjacent artery), bronchial wall thickening, tram-track opacities, mucus plugging, varicose or cystic bronchi
- CXR: tram tracks, ring shadows; less sensitive than HRCT
Other studies
- Spirometry — usually obstructive pattern; assess severity and trajectory
- Sweat chloride / CFTR testing in patients <40 or with suggestive features
- Ciliary studies (nasal nitric oxide, ciliary biopsy) if PCD suspected
- Bronchoscopy if foreign body, focal bronchiectasis, or atypical infection suspected
Diagnostic algorithm
| HRCT Feature | Description |
|---|---|
| Signet-ring sign | Internal bronchial diameter exceeds adjacent pulmonary artery diameter |
| Tram-track lines | Parallel linear opacities from thickened bronchial walls running peripherally |
| Cystic / varicose bronchi | Saccular dilations with or without air-fluid levels (severe disease) |
| Lack of bronchial tapering | Bronchi maintain caliber within 1 cm of pleura |
| Mucus plugging | Tree-in-bud or 'finger-in-glove' opacities |
Treatment
First-line
- Airway clearance — cornerstone of therapy: chest physiotherapy, oscillating PEP devices (Acapella, Flutter), high-frequency chest wall oscillation vest, active cycle of breathing
- Nebulized mucolytics/hydration: hypertonic saline (3-7%) inhalation; dornase alfa for CF only (may worsen non-CF bronchiectasis)
- Inhaled bronchodilator (albuterol) prior to airway clearance to ease maneuvers
- Treat exacerbations with prolonged antibiotics (10-14 days), targeted to prior sputum cultures: amoxicillin-clavulanate, doxycycline; ciprofloxacin if Pseudomonas
- Vaccinations: annual influenza, pneumococcal, COVID-19, RSV (age ≥60), Tdap
- Smoking cessation; pulmonary rehabilitation
Second-line / adjunct
- Chronic macrolide therapy (azithromycin 250-500 mg 3×/week) — reduces exacerbations (EMBRACE, BAT, BLESS trials); screen for QT prolongation and NTM before starting
- Inhaled antibiotics for chronic Pseudomonas: tobramycin, aztreonam, colistin (off-label in non-CF)
- Treat underlying cause: ABPA — itraconazole + steroids; CVID — IVIG; NTM — multidrug regimen; alpha-1 — augmentation
- Surgical resection (lobectomy) for focal disease with massive hemoptysis or refractory infection
- Bronchial artery embolization for life-threatening hemoptysis
- Lung transplantation for end-stage disease
Complications
- Recurrent exacerbations with progressive lung function decline
- Massive hemoptysis (bronchial artery hypertrophy)
- Chronic infection: Pseudomonas, NTM, Aspergillus colonization
- Cor pulmonale, respiratory failure
- Amyloidosis (chronic inflammation, rare)
PANCE pearls
- HRCT signet-ring sign — bronchial lumen larger than adjacent pulmonary artery — is the imaging hallmark of bronchiectasis.
- Always look for a reversible cause: CF testing in younger patients, immunoglobulins, alpha-1 level, ABPA workup, NTM, HIV.
- Pseudomonas isolation predicts more rapid lung function decline and worse prognosis; consider chronic inhaled antibiotic.
- Rule out NTM infection BEFORE starting chronic azithromycin — monotherapy in NTM-positive patients selects resistance.
- Sweat chloride ≥60 mmol/L is diagnostic of CF; 30-59 mmol/L intermediate (genetic testing needed); <30 mmol/L unlikely.
References
- BTS 2019 — British Thoracic Society Guideline for Bronchiectasis in Adults (Hill et al., Thorax 2019)
- ERS 2017 — European Respiratory Society Guidelines for the Management of Adult Bronchiectasis (Polverino et al., Eur Respir J 2017)
- EMBRACE Trial — Azithromycin for Prevention of Exacerbations in Non-CF Bronchiectasis (Wong et al., Lancet 2012)
- BLESS Trial — Long-term Erythromycin for Bronchiectasis (Serisier et al., JAMA 2013)
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