Pulmonary · PANCE / PANRE

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

HRCT findings diagnostic of bronchiectasis.

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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