Bronchietasis

Overview

•Post-infective - most common overall; measles, pertussis, TB, severe pneumonia

•Cystic fibrosis - most common identifiable cause in children in the UK; thick secretions overwhelm mucociliary clearance

•Primary ciliary dyskinesia (PCD) - AR disorder; dynein arm defects; Kartagener syndrome = dextrocardia + bronchiectasis + chronic sinusitis

•Immunodeficiency - humoral (IgG subclass deficiency, IgA deficiency, CVID, XLA); suspect in recurrent/severe pneumonias

•Airway obstruction - inhaled foreign body (young children), endobronchial tumour, lymph node compression → localised bronchiectasis

•ABPA - Aspergillus hypersensitivity in atopic/asthma/CF patients; causes proximal central bronchiectasis

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In any child with bronchiectasis, always investigate for CF, PCD, and immunodeficiency - sweat test, nasal NO, and immunoglobulins early.

Presentation

•Chronic productive cough - hallmark; daily mucopurulent sputum (>30 mL/day in severe disease)

•Recurrent chest infections - ≥3 LRTIs per year in children should prompt investigation

•Haemoptysis - up to 50-70%; usually small volume; massive haemoptysis >300 mL/24 h is a medical emergency

•Coarse inspiratory crackles - bibasal, do not clear completely with coughing

•Finger clubbing - moderate to severe disease

•Signs of underlying cause - dextrocardia (Kartagener), nasal polyps (CF/PCD), chronic rhinosinusitis (PCD)

Investigations

•Gold standard - HRCT chest - bronchial dilatation > adjacent pulmonary artery (signet ring sign), lack of tapering, bronchi within 1 cm of pleura, bronchial wall thickening

•Chest X-ray - tramline shadows, ring shadows; often normal in mild disease; insufficient alone to confirm/exclude

•Sputum culture - common pathogens: *Haemophilus influenzae* (most common), *Pseudomonas aeruginosa* (worse prognosis), *Staph aureus* (CF), *Moraxella catarrhalis*, NTM

•Spirometry - obstructive pattern (reduced FEV1/FVC); perform in children >5 years, monitor annually

•Immunoglobulins (IgG, IgA, IgM + IgG subclasses) - screen for humoral immunodeficiency

•Sweat chloride test - exclude CF; Cl >60 mmol/L diagnostic

•Nasal NO (nNO) - markedly reduced in PCD (typically <77 nL/min); screening test before formal ciliary studies

•Ciliary function studies (nasal brush biopsy - EM + high-speed video) - gold standard for PCD

•Aspergillus serology (total IgE, Aspergillus-specific IgE, precipitins) - screen for ABPA

•Bronchoscopy - single-lobe bronchiectasis in children to exclude inhaled foreign body

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The signet ring sign on HRCT: dilated bronchus (ring) appears larger than its accompanying pulmonary artery (signet stone) - normally they are roughly equal in size.

Management

•Airway clearance physiotherapy (ACBT, OPEP devices, postural drainage) - daily; twice daily during exacerbations; cornerstone of management

•Nebulised hypertonic saline - reduces sputum viscosity, augments clearance

•Acute exacerbation antibiotics (14 days, guided by culture):

•No *Pseudomonas* - oral amoxicillin or co-amoxiclav

•*Pseudomonas aeruginosa* - oral ciprofloxacin (if sensitive) or IV piperacillin-tazobactam / meropenem

•Bronchodilators (inhaled salbutamol or ipratropium bromide) - for demonstrable airflow obstruction

•Long-term suppressive antibiotics - ≥3 exacerbations/year or significant QoL impact; oral azithromycin three times weekly (also anti-inflammatory) or nebulised tobramycin/colistin for chronic Pseudomonas

•Mucoactive agents - nebulised dornase alfa recommended in CF-related bronchiectasis; evidence less clear in non-CF; nebulised hypertonic saline (6-7%) used in both

•Inhaled corticosteroids - not routinely recommended unless coexisting asthma or ABPA

•Surgical resection - focal single-lobe disease unresponsive to medical management; bronchial artery embolisation for massive haemoptysis

•Vaccinations - annual influenza + pneumococcal vaccine for all patients

Complications

•Massive haemoptysis - >300 mL/24 h; bronchial artery erosion; first-line is bronchial artery embolisation

•Respiratory failure - type 1 → type 2 in severe disease

•Cor pulmonale - chronic hypoxia → pulmonary hypertension → right heart failure

•Secondary (AA) amyloidosis - rare; chronic inflammation; presents with proteinuria and renal impairment

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Massive haemoptysis is a medical emergency. Position the patient with the bleeding side dependent. First-line: bronchial artery embolisation. Do NOT give physiotherapy during active haemoptysis.

Prognosis

•Pseudomonas aeruginosa colonisation - accelerated FEV1 decline, more frequent exacerbations, higher mortality

•Rheumatoid arthritis-associated bronchiectasis - particularly poor prognosis vs other non-CF bronchiectasis

•Early diagnosis and treatment of CF, PCD, or immunodeficiency in children can significantly slow disease progression