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Bronchiectasis

 

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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.

 

Click here for medical student OSCE and PACES questions about bronchiectasis

Common bronchiectasis exam questions for medical students, finals, OSCEs and MRCP PACES

 

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Perfect revision for medical students, finals, OSCEs and MRCP PACES