Key Facts

Bronchiectasis is characterised by permanent dilatation and thickening of the bronchial walls, resulting from a vicious cycle of infection, inflammation, and impaired mucociliary clearance. It is increasingly recognised as a common and debilitating chronic respiratory disease.

Epidemiology

• Prevalence: ~500-600 per 100,000 in the UK (increasing due to improved diagnosis with HRCT)
• More common in women and increasing with age
• Peak prevalence >70 years
• Significant burden on healthcare: frequent GP visits, antibiotic courses, hospital admissions

Aetiology

• Idiopathic (~50%): no identifiable cause after investigation
• Post-infective: childhood pneumonia, TB, whooping cough, measles, aspergillus
• Immunodeficiency: common variable immunodeficiency (CVID), IgA deficiency, HIV
• Cystic fibrosis: most common inherited cause
• ABPA: allergic bronchopulmonary aspergillosis (central bronchiectasis)
• Primary ciliary dyskinesia: Kartagener syndrome (situs inversus + bronchiectasis + sinusitis)
• Connective tissue disease: RA, Sjögren syndrome
• Inflammatory bowel disease: UC > Crohn
• Yellow nail syndrome: yellow nails + lymphoedema + pleural effusion + bronchiectasis
• Alpha-1 antitrypsin deficiency

Pathophysiology

Vicious cycle hypothesis (Cole):

• Initial insult (infection/inflammation) damages airways
• Impaired mucociliary clearance → mucus retention
• Bacterial colonisation and biofilm formation
• Chronic neutrophilic inflammation → further structural damage
• Progressive bronchial dilatation, destruction, and fibrosis
• Bronchial artery hypertrophy (risk of haemoptysis)

Typical Presentation

• Chronic productive cough with copious mucopurulent sputum (often daily)
• Recurrent chest infections (≥3/year)
• Breathlessness (progressive)
• Haemoptysis (mild to massive)
• Fatigue, reduced exercise tolerance

Clinical Signs

• Coarse inspiratory crackles (bilateral, lower zones often)
• Wheeze
• Finger clubbing (~3%)
• Sputum pot at bedside

Exacerbation Features

• Increased sputum volume and purulence
• Worsening breathlessness
• New or worsening cough
• Fever
• Deterioration in lung function

Red Flags

• Massive haemoptysis (bronchial artery erosion)
• Rapid decline in lung function
• New Pseudomonas isolation (associated with worse outcomes)
• Recurrent exacerbations (≥3/year)
• Progressive respiratory failure

Bedside

• Sputum MC&S: identify colonising organisms and guide antibiotic therapy
• Pulse oximetry: baseline

Bloods

• FBC: raised WCC (infection), eosinophilia (ABPA)
• CRP: elevated during exacerbations
• Immunoglobulins (IgG, IgA, IgM, IgE): screen for immunodeficiency
• Total IgE + Aspergillus-specific IgE and IgG: ABPA screen
• Alpha-1 antitrypsin level: if not previously tested
• Autoimmune screen: RF, anti-CCP, ANA (if rheumatological features)
• HIV test: if risk factors

Imaging

• HRCT chest: gold standard — bronchial dilatation (internal diameter > accompanying artery = signet ring sign), bronchial wall thickening, mucus plugging, tree-in-bud pattern, varicose/cystic changes
• CXR: tramlines, ring shadows; less sensitive than CT

Special Tests

• Spirometry: obstructive pattern (FEV₁/FVC <0.7); mixed in advanced disease
• Sputum mycobacterial culture: NTM screen
• Sweat test / CFTR genotyping: if CF suspected (<40 years, bilateral upper lobe, nasal polyps, male infertility)
• Nasal NO / ciliary biopsy: primary ciliary dyskinesia screen
• Bronchoscopy: if focal bronchiectasis (exclude foreign body, obstruction)
• CT sinuses: if PCD or ABPA suspected

Non-pharmacological

• Airway clearance techniques: physiotherapy-led; active cycle of breathing technique (ACBT), oscillatory PEP devices (Acapella, Flutter), postural drainage
• Pulmonary rehabilitation: for MRC dyspnoea ≥3 or functional limitation
• Annual influenza and pneumococcal vaccination
• Patient education and self-management plan

Pharmacological

Acute exacerbations:

• Empirical antibiotics guided by previous sputum cultures (14 days)
• First-line: amoxicillin 500mg TDS (or co-amoxiclav 625mg TDS if recurrent)
• Pseudomonas: ciprofloxacin 500-750mg BD PO or IV anti-pseudomonal (piperacillin-tazobactam)

Long-term management (BTS guideline):

• Mucolytics: carbocisteine 750mg TDS (or hypertonic saline nebulised 6-7%) to aid sputum clearance
• Long-term macrolide prophylaxis: azithromycin 250mg 3×/week or 500mg 3×/week (if ≥3 exacerbations/year)
  • Check baseline ECG (QTc prolongation risk), LFTs, NTM sputum culture
  • EMBRACE, BAT, BLESS trials: reduced exacerbation frequency by 40-50%
• Pseudomonas eradication: first isolation — ciprofloxacin 500-750mg BD for 2 weeks ± nebulised colistin/gentamicin for 3 months
• Long-term nebulised antibiotics: colistin 1-2 MU BD or gentamicin 80mg BD (chronic Pseudomonas with frequent exacerbations)
• Inhaled bronchodilators: if airflow obstruction

Surgical/Interventional

• Bronchial artery embolisation: for massive/recurrent haemoptysis
• Surgical resection (lobectomy): localised disease with recurrent severe infections, refractory to medical therapy
• Lung transplantation: end-stage bilateral bronchiectasis (particularly CF-related)

Referral Criteria

• Respiratory specialist referral for all new diagnoses of bronchiectasis
• Specialist input for Pseudomonas colonisation, frequent exacerbations, or diagnostic uncertainty
• Immunology referral if immunodeficiency confirmed
• Thoracic surgery for consideration of resection in localised disease

• Variable depending on aetiology, microbiology, and exacerbation frequency
• Pseudomonas colonisation: 3-fold increased mortality and accelerated lung function decline
• Frequent exacerbations (≥3/year): associated with worse QoL and survival
• BSI (Bronchiectasis Severity Index) predicts 4-year mortality: mild 5.3%, moderate 9.9%, severe 29.3%
• Long-term macrolide prophylaxis reduces exacerbation frequency by 40-50%
• CF bronchiectasis: median survival now ~50+ years with modern therapies
• Non-CF bronchiectasis: life expectancy generally good with appropriate management

Bronchiectasis Severity Index (BSI)

Aetiology Investigation Checklist