Bronchiectasis
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
- To treat exacerbations refer to local guidelines but examples include:
- 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
- Antibiotics
- Surgical
- Resection in localised disease
- Lung transplant (heart/lung transplant)
- Bronchial artery emobolisation or surgery for management of haemoptysis
- Resection in localised disease
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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