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Definition of bronchiectasis

  • Abnormal and permanent dilatation of airways
    • Bronchial walls become inflamed, thickened and irreversibly damaged
    • The mucociliary elevator is impaired
    • Mucus accumulates leading to increased susceptibility to infection


 Epidemiology of bronchiectasis

  • Prevalence in the UK estimated as 100/100,000
    • Prevalence increases with age


 Causes of bronchiectasis

  • Congenital
    • Cystic Fibrosis
    • Primary Ciliary dyskinesia (sinusitis, bronchiectasis and azospermia)
    • Kartagener’s  (primary ciliary dyskinesia with dextrocardia and situs inversus)
    • Young’s syndrome (azospermia and sinusitis)
    • Pulmonary sequestration
  • Mechanical obstruction
    • Foreign body
    • Bronchial carcinoma
    • Post-TB Stenosis
    • Lymph node
  • Post-Infective
    • Measles
    • TB
    • Pertussis
    • Bacterial and viral pneumonia
  • Granulomatous Disease
    • TB
    • Sarcoidosis
  • Usual interstitial pneumonia (cryptogenic fibrosing alvelolitis)
  • Immune over-activity
    • Allergic broncho-pulmonary aspergillosis (ABPA)
    • Inflammatory bowel disease
    • Rheumatoid arthritis
    • Sjorgrens
    • Post lung transplant
  • Immune deficiency
    • Hypogammaglobulinaemia
    • Selective immunoglobulin deficiencies (IgA and IgG2)
    • Secondary
      • HIV, malignancy
  • Aspiration
    • Chronic alcoholics
    • GORD


 Presentation of bronchiectasis

  • Symptoms
    • Cough
    • Shortness of breath
    • Excessive sputum production
    • Recurrent chest infections
    • Haemoptysis
  • Signs
    • Cachexia and lymph nodes
    • Clubbing
    • Hyperinflation
    • On Auscultation
      • Coarse crackles in affected areas: mixed character, alter with coughing
      • Squeaks and Wheeze
      • Inspiratory clicks


 Differential diagnosis of bronchiectasis

  • Pulmonary fibrosis
  • Bronchial carcinoma
  • Chronic lung abscess
  • Asbestosis


 Investigation of bronchiectasis

  • Sputum culture and cytology
  • CXR
    • Tramlines and ring shadows. Bullae.
  • HRCT
    • “Signet ring” sign: thickened, dilated bronchi larger than the adjacent vascular bundle
  • Sinus x-rays
    • 30% have concomitant sinusistis
  • Spirometry
    • Normal/ restrictive picture
  • For a specific cause:
    • Bronchoscopy
    • Immunoglobulins
    • Aspergillus RAST and skin prick testing
    • Sweat electrolyte test
    • Mucocilliary clearance
      • Nasal saccharine taste test: 1mm cube of saccharine placed on inferior turbinate should be tasted within 30mins


 Management of bronchiectasis

  • Non-pharmacological
    • Multidisciplinary team (MDT) input
    • Physiotherapy
      • Postural drainage
      • Active cycle breathing
    • Smoking cessation
    • Immunisations
  • Medical
    • Antibiotics
      • To treat exacerbations refer to local guidelines but examples include:
        • Amoxicillin 500mg tds or clarithromycin 500mg bd for 2 weeks as 1st line
        • Ciprofloxacin in pseudomonas colonisation
        • High dose maybe needed in severe bronchiectasis with Haemophilus infuenzae B colonisation e.g. amoxicillin 1g tds
    • Long term antibiotics
      • Consider in patients having ≥ 3 exacerbations per year or patients with fewer exacerbations causing significant morbidity e.g. low dose azithromycin three times per week
      • Inhaled antibiotics can also be used
    • Bronchodilators/ inhaled corticosteroids if there is any evidence of airflow obstruction
    • Inhaled Saline
    • NIV/ Intermittent positive pressure may be used to augment tidal volume and reduce work of breathing
  • Surgical
    • Resection in localised disease
      • Lung transplant (heart/lung transplant)
      • Bronchial artery emobolisation or surgery for management of haemoptysis


 Complications of bronchiectasis

  • Progressive respiratory failure
  • Cor pulmonale
  • Pneumonia
  • Pneumothorax
  • Empyema
  • Life-threatening haemoptysis: Mycotic aneurysm (esp. in patients with CF)
  • Secondary amyloidosis


 Prognosis of bronchiectasis

  • Vastly improved with antibiotic therapy, but most still eventually progress to respiratory failure due to chronic damage.


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