Bronchiectasis

• Bronchiectasis involves permanent dilation of the bronchi

  Initial insult to the bronchi results in recruitment of immune cells that damage the muscle and elastin
• Dilated bronchi are predisposed to persistent microbial colonisation as mucus is trapped
• Causes of bronchiectasis:

  • Post infection (most common): recurrent childhood LRTIs, pulmonary TB and allergic bronchopulmonary aspergillosis
  • Pulmonary disease: COPD and asthma
  • Congenital: Cystic fibrosis and alpha-1 antitrypsin deficiency
  • Connective tissue disorders: RA, SLE and sarcoidosis
  • 40% of cases are idiopathic
• Key presenting symptoms are:

  • Daily cough productive of large amounts of mucopurulent sputum
  • Haemoptysis is present on 50% of patients
  • Exertional dyspnoea which may progress to resting dyspnoea
  • Fatigue
  • Rhinosinusitis symptoms - nasal discharge, nasal obstruction and facial pressure
• Bronchiectasis on examination:

  • Finger clubbing - increased secretion of growth factors
  • signs of connective tissue disease
  • Course crepitations present on inspiration and expiration that change or clear when coughing
  • Rhonchi - low pitched snoring noise on auscultation
  • High pitched inspiratory speaks and pops
• Typical pulse oximetry target is 94-98%
• Investigations:

  • Sputum culture: most commonly pseudomonas aeruginosa or haemophilus influenzae
  • Spirometry - obstructive pattern but may be normal
  • Echo - bronchiectasis may impair ventricular function and lead to pulmonary hypertension
  • FBC - raised white blood cells
  • Autoimmune screen - Anti-CCP, ANA and ANCA
  • IgE
  • Genetic testing - cystic fibrosis
• Bronchiectasis can cause signs of cor pulmonale such as a raised JVP
• Advanced bronchiectasis will show tram lines and ring shadows on a chest x-ray

  HRCT is the gold standard imaging test - bronchial dilation with or without airway thickening
• General management for bronchiectasis:

  • Pulmonary rehab - airways clearance techniques
  • Smoking cessation
  • Vaccinations
• Medical management of bronchiectasis:

  • Mucoactive agents - nebulised saline and carbocisteine
  • Long term antibiotics if there are frequent exacerbations - azithromycin
  • LABAs such as formoterol for dyspnoea
  • LTOT
  • Lung resection or transplant
• Risk factors:

  • Age over 70
  • Female gender
  • Smoking history
• Important areas to cover in the history:

  • History of childhood lower respiratory tract infections
  • Family history - congenital conditions such as cystic fibrosis and autoimmune conditions such as RA
  • Smoking history
• Disease-related complications of bronchiectasis include:

  • Respiratory failure: due to failure of gas exchange in the lungs
  • Massive haemoptysis (>250ml per day): often due to rupture of a bronchial artery into a bronchus
  • Anxiety and depression: due to impaired quality-of-life
• Treatment-related complications of bronchiectasis include:

  • Azithromycin - long QT syndrome, tinnitus and hearing loss
  • Lung transplant: immediate complications (e.g. blood loss), early complications (e.g. transplant rejection) and late complications (e.g. post-transplantation lymphoproliferative disorder)
• Haemophilus influenzae is the most common organism