A 56-year-old woman presents to her GP with a five-year history of a chronic cough that is productive of purulent sputum. She has required two to three courses of oral antibiotics per year over the past few years, and sputum samples have previously cultured Haemophilus influenzae on several occasions. She suffered with whooping cough as a child but has never smoked. She has early finger clubbing and left-sided coarse inspiratory crackles. Her chest X-ray is reported to be normal. Despite this, the GP suspects an underlying diagnosis of bronchiectasis and refers her to the respiratory clinic.
Bronchiectasis results from an event or series of events that causes damage to the airways, resulting in persistently dilated thick-walled bronchi. People with bronchiectasis typically present with a chronic productive cough, which may be associated with additional symptoms of breathlessness, chest pain and haemoptysis and can progress to respiratory failure and cor pulmonale.
A targeted history should be obtained, enquiring as to conditions that are associated with bronchiectasis.
Cystic fibrosis should also be considered in children and younger adults, particularly in the presence of a family history, male infertility or idiopathic pancreatitis.
Factors that suggest a diagnosis of bronchiectasis include a chronic daily cough productive of purulent sputum, requirement for frequent systemic antibiotics and persistently positive sputum cultures for airway pathogens.
Findings on examination include crackles, which are most common in the lower zones. Patients may also have wheeze and finger clubbing.
Although bronchiectasis may be evident on chest X-ray, high-resolution CT thorax (HRCT) is the radiological investigation of choice to confirm the diagnosis and assess the extent of disease. HRCT may also suggest an underlying cause such as allergic bronchopulmonary aspergillosis (ABPA). People confirmed to have bronchiectasis should be investigated for an identifiable underlying cause, but even with extensive testing a cause is not found in up to 40% of cases.
Underlying causes of bronchiectasis include:
• Immune deficiency.
• Cystic fibrosis (CF).
• Primary ciliary dyskinesia.
• Inflammatory bowel disease.
• Repeated gastric aspiration.
• Yellow nail syndrome.
• No cause identified (40% of cases).
A bronchiectasis screen includes full blood count, serum immunoglobulins, serum electrophoresis, IgE to Aspergillus fumigatus and Aspergillus precipitins. If clinically relevant, additional testing may be performed to exclude CF, connective tissue disease, vasculitis, ciliary disorders and additional immunological disorders. Sputum samples should be tested for microscopy, culture and sensitivities at diagnosis and prior to starting antibiotics for exacerbations. Sputum should be tested for acid-fast bacilli in the presence of unexplained persistent fevers, sweats or weight loss, as well as in patients not improving on routine antibiotics. Lung function tests – FEV1, FVC and peak flow – should be performed at diagnosis and repeated at least annually.
A personalised self-management plan should be provided, advising patients on symptoms that should prompt antibiotic therapy and when to seek medical attention. All patients with bronchiectasis should be taught airway clearance techniques by a physiotherapist. Patients may benefit from additional treatments to improve sputum clearance, such as mucolytics, nebulised hypertonic saline or carbocysteine. Patients with reversible airflow obstruction may benefit from inhaled bronchodilators.
Antibiotics should be given for exacerbations associated with worsening symptoms (cough, increased sputum volume or change of viscosity, increased sputum purulence with or without increasing wheeze, breathlessness, haemoptysis) or systemic upset. Patients typically require 14 days of oral antibiotics and the antibiotic choice should ideally be guided by previous sputum microbiology. In the absence of previous microbiology, first-line treatment is amoxicillin 500mg tds (or clarithromycin 500mg bd in penicillin allergy). Higher-than-usual doses (such as amoxicillin 1g tds) may be required in patients with severe disease. Ciprofloxacin 500-750mg bd should be used in patients chronically infected with Pseudomonas aeruginosa, although intravenous antibiotics are often required to achieve a clinical improvement in patients with this organism. Criteria for hospital admission include inability to cope at home, cyanosis, confusion, breathlessness with respiratory rate >25 breaths per minute, respiratory failure, circulatory failure, fever >38°C, inability to take oral medications and failure to improve after oral antibiotics.
Long-term oral antibiotics, or rotational (cycling) antibiotics may be considered to reduce exacerbation frequency in selected patients. Daily low-dose azithromycin (250mg od or 500mg three-times weekly) has been shown to reduce frequency of exacerbations, but there is a potential for the development of increased bacterial resistance.1 Long-term nebulised antibiotics may be used in patients with chronic P. aeruginosa infection.
Patients with non-CF bronchiectasis who need secondary care follow-up include those with:2
- Chronic P. aeruginosa, MRSA or non-tuberculous mycobacterial infection
- Declining lung function or frequent exacerbations
- Receiving prophylactic antibiotics
- Immune deficiency
- Connective tissue disease
- Inflammatory bowel disease
- Primary ciliary dyskinesia
- Advanced disease or considering transplantation.
Dr Edward Nash is a consultant in respiratory medicine at Heart of England NHS Foundation Trust, Birmingham
1 Altenburg J, de Graaff CS, Stienstra Y et al. Effect of azithromycin maintenance treatment on infectious exacerbations among patients with non-cystic fibrosis bronchiectasis: the BAT randomized controlled trial. JAMA 2013;309:1251-9.
2 Pasteur MC, Bilton D, Hill AT. British Thoracic Society guideline for non-CF bronchiectasis. Thorax 2010;65 Suppl 1:i1-58.