Bronchiectasis is a chronic respiratory disease characterised by permanent bronchial dilation, due to irreversible damage to the bronchial wall.1
In the UK, the prevalence of bronchiectasis is 3 per 1000, and 60% of diagnoses are in patients over the age of 70. The prevalence of bronchiectasis in the UK has increased by 60% over the last 20 years.2,3
Patients with mild bronchiectasis often have normal life expectancies. The mortality risk increases in patients with more frequent exacerbations and worse lung function.4
The pathogenesis of bronchiectasis is poorly understood. An initial insult to the bronchi (e.g. infection) results in immune cells being recruited to the bronchi. These immune cells secrete cytokines and proteases, leading to inflammation in the bronchi.
This inflammation damages the muscle and elastin found in the bronchial walls, leading to bronchial dilation.
In most people, this bronchial dilation is reversible after the resolution of the initial insult to the bronchi. However, in patients with bronchiectasis, several factors prevent the bronchial dilation from reversing (e.g. impaired mucociliary clearance and dysregulated immunity).7
Dilated bronchi are predisposed to persistent microbial colonisation, as mucus traps in the dilated bronchi (figure 1).
Therefore, bronchiectasis patients get caught in a vicious cycle, whereby their airways are colonised by micro-organisms, which increases bronchial inflammation, worsening their bronchiectasis, leading to increased susceptibility to airway colonisation.8
Causes of bronchiectasis
There is a wide range of possible underlying causes of bronchiectasis, which are summarised below.
More acute presentation (over days, rather than months or years)
Chest X-ray: consolidation
Vesicular breath sounds
Radiological investigations: normal
Relevant bedside investigations include:
Pulse oximetry: aim for 94-98% initially, but the target saturations may be reduced in advanced disease (this is a risk-benefit judgement made by a senior clinician)
Sputum culture: common organisms isolated include Pseudomonas aeruginosa and Haemophilus influenza. Pseudomonas is particularly common, as it forms biofilms which protect it from the immune system and antibiotics.14
Lung function tests: typically show an obstructive pattern (FEV1/FVC ratio < 70%), but may be normal
Echocardiogram: bronchiectasis may impair ventricular function and lead to pulmonary hypertension15
Relevant laboratory investigations include:
Full blood count: may show elevated white blood cell count, including neutrophilia
CRP: may be elevated during acute exacerbations
Autoimmune screen (if suspecting an autoimmune condition): includes anti-CCP, ANA and ANCA
Specific IgE to Aspergillus fumigatus: if suspecting ABPA
Genetic testing (done in specialist units): to diagnose congenital disorders, such as cystic fibrosis and primary ciliary dyskinesia
Relevant imaging investigations include:
Chest X-ray: required to exclude other pathologies. Chest X-ray may be normal in mild bronchiectasis. Signs in severe disease include tram lines and ring shadows.
High-resolution CT chest (figure 3): gold-standard imaging test. Shows bronchial dilation, with or without airway thickening.16
Bronchoscopy (figure 4): used in patients with localised bronchiectasis, as this may be caused by foreign body aspiration or an endobronchial lesion.17
Conservative management options for bronchiectasis include:
Pulmonary rehabilitation: refer the patient to a respiratory physiotherapist, who will teach the patient airway clearance techniques (which offload mucus from the airways)
Smoking cessation: see the Geeky Medics guide on smoking cessation
Annual influenza vaccination and one-off pneumococcal vaccination
Medical management options for bronchiectasis include:
Mucoactive agents (e.g. nebulised saline and carbocisteine): aid the clearance of sputum, for patients who have difficulty expectorating sputum (such as frail, elderly patients)17
Long-term antibiotics (e.g. azithromycin three times a week): may be used in patients who have three or more exacerbations per year, after consultation with a respiratory specialist1
Bronchodilators: offer a long-acting bronchodilator (e.g. formoterol) in patients with activity-limiting dyspnoea
Specific treatments for underlying conditions: CFTR modulator therapies (e.g. Trikafta) can be used in cystic fibrosis
Long-term oxygen therapy: if saturations on room air are <88% or PaO2 on room air is <7.3kPa
Surgical management options for bronchiectasis include:
Lung resection: for localised bronchiectasis, not controlled by optimum medical management
Lung transplant (figure 5): for patients younger than 65, with rapid deterioration despite optimum medical management17
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 bronchus21
Anxiety and depression: due to impaired quality-of-life
Treatment-related complications of bronchiectasis include:
Macrolides: 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)
Bronchiectasis is a chronic respiratory disease characterised by permanent bronchial dilation, due to irreversible damage to the bronchial wall.
Bronchiectasis is commonly caused by respiratory infections, but can be caused by systemic conditions.
The main symptoms are chronic productive cough (with mucopurulent sputum) and dyspnoea.
A diagnosis of bronchiectasis is based on radiological findings, with high-resolution CT chest being the gold-standard diagnostic investigation.
Management includes respiratory physiotherapy, mucoactive agents and long-term antibiotics.
The main complication of bronchiectasis is respiratory failure.
Dr Neeraj Shah
Respiratory medicine registrar
Dr Chris Jefferies
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