Originally published: November 2018

Key learning points

  • Bronchiectasis is a chronic condition characterised by irreversible airway dilatation
  • Patients with bronchiectasis usually present with chronic cough with sputum production
  • Other common symptoms are recurrent respiratory infections, dyspnoea, wheeze, fatigue and sometimes haemoptysis
  • Bronchiectasis can develop as a primary disease but also as a complication of other lung diseases such as COPD or asthma and non-respiratory diseases such as rheumatoid arthritis
  • A high-resolution CT chest scan should be used to confirm a diagnosis
  • Management should aim to prevent exacerbations, reduce symptoms and improve quality of life, while minimising progression of the disease
  • Antibiotics can be used to manage exacerbations or long-term to suppress microbial load and reduce further symptoms and exacerbations

Introduction

Bronchiectasis is a chronic lung condition that is characterised by irreversible airway dilatation.1 Patients usually present with chronic cough and sputum production,1 with other common symptoms being breathlessness, wheeze, fatigue and, sometimes, haemoptysis.2–4

The severity of the condition varies enormously, with some patients having few symptoms and little impact on their daily life.5 In more severe disease, however, patients will often experience persistent lower airway bacterial infections (or exacerbations), leading to recurrent hospital admissions, reduced quality of life and increased risk of mortality.1,2,5–7

Bronchiectasis seems to be becoming more common, particularly in older people, and higher rates are found in women.6

The vicious cycle hypothesis describes how a combination of bronchial infection, inflammation and impaired mucociliary clearance leads to progressive airway damage and dilatation, resulting in symptoms and exacerbations.7

Causes

In up to 50% of patients there is no obvious underlying cause, known as idiopathic bronchiectasis, which usually has an onset later in life.4,6,8 However, the condition may follow a previous infection or may be linked to a number of other conditions (see Box 1).

Box 1: Some conditions that may be associated with bronchiectasis4–7,9

Previous severe infection: TB, pneumonia, pertussis, non-tuberculous mycobacteria, influenza, HIV, staphylococcal or mixed infection
Airways diseases: COPD, asthma, allergic bronchopulmonary aspergillosis
Inflammatory conditions: Rheumatoid arthritis, inflammatory bowel disease
Bronchial obstruction: Tumour, foreign body, mucus impaction
Aspiration: Gastro-oesophageal reflux disease, after lung transplantation
Congenital or hereditary conditions: Mounier-Kuhn syndrome, Young's syndrome, immunodeficiency, for example, primary ciliary dyskinesia or immunoglobulin deficiency

It is important to identify any underlying conditions to guide management and rule out causes that require specific targeted therapies, such as cystic fibrosis and immunodeficiency.9 Investigations to identify associated conditions should include genetic testing for cystic fibrosis, Aspergillus precipitins, immunological profile and sputum microbiology.9

Diagnosis

Patients often experience long delays before receiving a diagnosis because the symptoms are common and mimic other respiratory diseases. Patients should be suspected of having bronchiectasis if they have a persistent cough, sputum production and/or recurrent respiratory symptoms.7 Children should be investigated if they have asthma that is hard to treat.5

The most common clinical sign is persistent crackles heard on auscultation.5,9

Sputum purulence and volume should be recorded, along with the number of respiratory infections in the previous year,9 and sputum microbiology should be carried out to identify any underlying pathogens.5

Chest X-rays may be normal but should be used to rule out other conditions such as lung cancer.5

Spirometry can confirm chronic airflow limitation (airways obstruction) but bronchiectasis can occur in the presence of normal spirometry or even a restrictive pattern.5

A high-resolution CT (HRCT) chest scan should be considered to confirm the diagnosis.9 HRCT images will show the characteristic feature of bronchial wall dilatation and bronchial wall thickening will often be present.9

A scoring system, such as the bronchiectasis severity index, can be used to define the severity and risk of exacerbations.1

Management

A multidisciplinary approach is key to management, which should aim to prevent exacerbations, reduce symptoms and improve quality of life, while also minimising progression of the disease.7 Patient education is important, so that patients can manage their condition and know how to recognise exacerbations; ideally they should be involved in creating personalised self-management plans.

There are many elements to managing this complex disease:5,7,9

  • Daily physiotherapy is needed to clear the airways, with patients encouraged to do this independently wherever possible
  • Simple exercise can help to clear lungs, maintain fitness and reduce exacerbations
  • Pulmonary rehabilitation should be offered to patients who have significant problems with breathlessness
  • In selected cases it may be appropriate to treat airway obstruction and wheeze with bronchodilators
  • Vaccinations should be offered for influenza and Streptococcus pneumoniae
  • Comorbidities common in patients with bronchiectasis including anxiety and depression, urinary incontinence, sexual dysfunction and nutritional deficiencies, should be managed as part of the multidisciplinary approach
  • Surgery is the only curative treatment where resection of a single chronically infected lobe can sometimes be of benefit for patients with localised disease that cannot be managed with medical treatment

Antibiotic treatment

Antibiotics are a key element of bronchiectasis management. They can be used acutely to manage exacerbations and chronically to suppress microbial load with the aim of reducing symptoms and exacerbations. As yet, however, we do not have good biomarkers to direct when to start (and stop such therapies). Sputum purulence varies widely between patients and can vary across different times of the day. Often a persistent increase over 48 hours in sputum purulence associated with symptoms of increased cough, increased sputum volume associated with breathlessness or increased wheeze serves as a trigger for starting exacerbation management therapy. British Thoracic Society and European Respiratory Society guidelines recommend 14 days of oral antibiotics guided by prior sputum culture data.7,9

Long-term macrolides are not licensed for use in bronchiectasis but some patients do receive these. Three large randomised controlled trials have shown a benefit, with reductions in exacerbations and sputum production, but they were all associated with significant macrolide resistance.10–12 Their use should therefore probably be reserved for patients in secondary care follow-up who still have frequent exacerbations despite optimisation of other modalities such as physiotherapy. Side effects may include deafness and caution needs to be made in those with abnormal cardiac conduction due to QT prolongation so a baseline ECG should be obtained.10,12

Long-term nebulised antibiotics are not specifically licensed in bronchiectasis but are recommended in the guidelines.9 Again these are used only in selected cases after optimisation of other therapies. Agents that have been used include nebulised colistin and nebulised gentamicin.9

Referral

Most cases of bronchiectasis should be referred to secondary care to specific clinics with expertise in the condition. Patients that require prompt referral are those with more severe disease, persistent Pseudomonas isolation, haemoptysis, more than two exacerbations in a year and those with atypical features such as severe disease at a young age.

Dr Hayley Mainman is a respiratory SpR, Newcastle-upon-Tyne

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