Bronchiectasis

• What is bronchiectasis?

  Bronchiectasis is a chronic lung condition characterised by the widening and inflammation of the bronchial tubes.

  Irreversible dilated bronchi with chronic bronchial inflammation & infection
• Bronchiectasis is commonly associated with Hx of severe lower resp tract infection.
• Pathophys of bronchiectasis


  Vicious cycle hypothesis (describes bronchiectasis as arising from a combination of genetic & environmental factors)

  • Deficit in mucociliary clearance +/- immune function → microorganism acquisition, colonisation & infection → chronic inflammation → dilation & thickening of bronchi → bronchial wall oedema & increased mucous production → more infections → further inflammation & damage
• What are the 3 morphological types of bronchiectasis?


  Tubular/cylindrical

  Varicose

  Cystic
• What is tubular/cylindrical morphology of bronchiectasis?


  Most common types

  Morphology → smooth uniform dilatation of bronchi with loss of normal tapering

  Associated with ‘signet ring sign’ (due to increased bronchoarterial ratio) & ‘tram-track sign’ (due to parallel bronchial walls)
• What is varicose morphology of bronchiectasis?


  Relatively uncommon

  Bronchi are irregular with dilatation interspersed with areas of constriction
• What is cystic morphology of bronchiectasis?


  Associated with cystic fibrosis

  Dilated bronchi that are cyst like & filled with air/fluid
• What are the investigations for bronchiectasis?


  Sputum culture

  CXR (majority will be abnormal but non-specific)

  Post-bronchodilator spirometry (commonly shows obstructive pattern (can be mixed, restrictive or obstructive))

  High resolution CT

  FBC

  O2 sats

  Serum total IgE

  Assessment of sensitisation to Aspergillus fumigatus

  Serum IgG, IgA & IgM (Inx for immunodeficiency)
• What are the indicative signs of bronchiectasis on CT


  Bronchoarterial ratio > 1

  • Internal airway lumen is larger than adjacent pulmonary artery (signet ring sign)

  Lack of tapering

  • Bronchi should taper in diameter as they travel distally from the lung hila to the periphery

  Bronchus visible within 1cm of pleural surface

  • Normal, non-dilated airways cannot usually be seen within 2cm of the pleura
• What are the DDx of bronchiectasis?


  COPD

  Asthma

  Lung cancer

  TB

  Rhinosinusitis

  GORD
• How can COPD be differentiated from bronchiectasis?


  Sputum is more likely to be clear in COPD (except during infective exacerbation)

  Smoking Hx is strongly associated with COPD

  NOTE: Conditions may co-exist
• How can asthma be differentiated from bronchiectasis?


  Episodes of dyspnoea & cough may be associated with particular triggers (exercise, allergens etc)

  Cough is more likely to be dry in asthma
• How can lung cancer be differentiated from bronchiectasis?


  Presence of weight loss & hoarse voice in lung cancer
• How can TB be differentiated from bronchiectasis?


  Presence of weight loss & night sweats in TB
• What are the RFs of bronchiectasis?


  Cystic fibrosis

  Host immunodeficiency

  Previous infections -> associated with bronchial destruction

  Congenital disorders of bronchial airways

  Primary ciliary dyskinesia
• What is primary ciliary dyskinesia?


  Autosomal-recessive disorder with a defect in ciliary structure and/or function

  Dysfunctional cilia lead to recurrent ear, sinus, and lung infections
• What are the symptoms of bronchiectasis?


  Persistent productive cough

  Dyspnoea

  Fatigue

  Fever

  Haemoptysis

  Rhinosinusitis symptoms - nasal discharge/blockage/congestion, facial pain/pressure, reduced sense of smell

  Chest pain

  Weight loss

  Recurrent chest infections
• What are the signs of bronchiectasis?


  Coarse crackles

  Wheeze

  High-pitched inspiratory squeaks

  Large airway rhonchi (low pitched snore-like sounds)

  Palpable chest secretions on coughing or forced expiratory manoeuvre (persisting over time)

  Clubbing
• What are the possible complications of bronchiectasis?


  Respiratory failure

  Pulmonary infections

  Haemoptysis

  Pulmonary HTN & cor pulmonale

  GI issues

  Osteoporosis
• How is resp failure a complication of bronchiectasis?


  Hypoxaemia & hypercapnia may develop → due to ventilation-perfusion mismatch → resp failure

  Common complication in advanced disease
• How are pulmonary infections a complication of bronchiectasis?

  Abnormal dilatation of bronchi → predisposes to recurrent lower resp tract infection

  Common pathogens -> pseudomonas aeruginosa, H. influenzae, S. pneumoniae
• How is haemoptysis a complication of bronchiectasis?


  Chronic inflammation & infection → erosion of the bronchial arteries → haemoptysis (can range from mild to life-threatening)
• How are pulmonary HTN & cor pulmonale complications of bronchiectasis?


  Chronic hypoxia → pulmonary HTN → may eventually lead to R-sided HF or cor pulmonale
• How are GI issues complications of bronchiectasis?


  Malabsorption & weight loss → may occur due to increased caloric demand from chronic coughing & clearance of secretions
• What is the initial management of bronchiectasis?


  Identify & treat underlying cause

  Airway clearance techniques +/- pulmonary rehabilitation (AIM: loosen & clear mucous, improve ventilation & cough efficiency)

  Annual Influenza vaccine

  Abx for exacerbations (7-14 days) -> choice based on sputum culture

  Self-management plan
• What is the criteria lung transplant in bronchiectasis?


  Age ≤ 65 years

  FEV < 30% predicted with significant clinical instability

  Rapid deterioration despite optimal medical management.