Bronchiectasis
Pathophysiology
• Chronic irreversible dilatation of bronchial tree
○ Chronic/recurrent infection leads to permanent dilatation. This causes poor mucous clearance which again causes infection
○ Cycle leading to deterioration
○ Nb no effect on parenchyma and is a disease of the airways
○
• Certain organisms cause the infections
○ Pneumococcus
○ S Aureus
○ H influenzae
○ Pseudomonas
Aetiology
• All 3 factors in the cycle have different causes
○ Post-infectious – Can be a complication of certain infections
§ Measles
§ Pertussis
§ Bronchiolitis
§ Pneumonia
§ TB
○ Congenital
§ Cystic Fibrosis
§ Ciliary dysfunction syndromes – Kartagener’s or Primary Ciliary Dyskinesia
§ Young’s Syndrome
○ Bronchial obstruction
§ Malignancy – e.g. bronchogenic carcinoma
§ Chronic aspiration
§ Long-standing foreign body
○ Immunodeficiency
§ Hypogammaglobinaemia conditions – e.g. CVID, X-linked agammaglobulinaemia
○ Iatrogenic – Bronchial aspiration and mechanical obstruction
Clinical features:
• Symptoms
○ Chronic cough with copious purulent sputum production
§ Worse in mornings
§ Worse with changes in posture
○ Recurrent intermittent haemoptysis
§ May be massive with a haemorrhage
○ Halitosis
○ Recurrent infections
§ Pseudomonas is a key pathogen but others also occur
§ Sepsis and other signs of infection
• Signs
○ Resp
§ Coarse crackles which are removed by coughing
§ Wheeze
§ Purulent sputum
§ Clubbing
○ Systemic
§ Weight loss
•
Complications
• Pneumonia
• Pleural effusion
• Pneumothorax
• Pulmonary HTN
• Haemorrhage => Massive haemoptysis
• Cerebral abscess
• Amyloidosis
Investigations
• Generally similar to infections
○ BOBIS
• Orifices
○ Sputum MC&S – Can identify the pathogen
○ Throat swab
• Bloods
○ Routine + CRP
○ Check serum Igs
○ Blood cultures
○ ABG – T1RF as with other infections
• Imaging
a. CXR
§ Tramlines and rings
§ May have consolidation
b. High-Res CT can be used to further investigate the extent and distribution of disease
§ Show bronchial wall thickening -> signet ring sign
• Special
a. Spirometry
§ Shows an obstructive pattern
§ Checking for reversibility can be useful to determine potential treatments
b. Bronchoscopy
§ Identify the area of haemoptysis
§ Biopsy can also be taken => analysis for CF etc
Management
• All patients need education
○ Annual follow-up is required
ABC
• ABC approach – treat the symptoms
○ Similar to acute COPD exacerbation
1. Oxygen and NIV may be required
2. Nebulised Bronchodilators
○ Nebulised salbutamol is used
Acute treatment
1. Nebulised Corticosteroids are needed to reduce inflammation
2. Antibiotics – target the bacteria according to sensitivities
○ Can be given oral, inhaled, nebulised, or IV
Long-term
1. Mucous drainage
○ Chest Physiotherapy
§ Expectoration techniques
§ Postural drainage – done twice daily
○ Mucolytic agents
§ Carbocysteine
2. Preventative long-term antibiotics
○ are given if >3 exacerbations in one year
○ Can also be given oral, inhaled, nebulised, or IV
3. Treat underlying cause
a. E.g. DNAase for CF
b. E.g. IVIg for immunodeficiency
4. Surgery is rarely used – in severe but localised disease or to control haemoptysis