Bronchiectasis can be an illness in itself, or it may be a part of another condition such as cystic fibrosis (CF).
It can be diagnosed either by pathology, or by a high resolution CT scan of the chest.
Bronchiectasis can occur as a result of an infective insult, with symptoms such as impaired clearance mechanisms, local obstruction, impaired local structural defences or defective immune responses.
The process may be localised to part of a lung, or it may be found throughout the lungs.
Even with thorough investigation, about 50 per cent of cases of bronchiectasis remain of idiopathic aetiology.
The patient may present at any age. They usually exhibit signs of a persistent cough, which is productive of mucopurulent sputum, throughout the year.
Patients are likely to be treated for frequent chest infections or bronchitis before the final diagnosis is made.
The presence of these symptoms in a non-smoker should make us question a diagnosis of bronchitis.
Exacerbation presents with the features of chest infection such as cough, dark sputum, haemoptysis, fever, pleuritic chest pain and wheeze.
Patients presenting in adulthood may have a history of multiple previous infections or wheezy bronchitis in childhood. Many patients will also complain of chronic tiredness.
Examination may reveal the classic features of finger clubbing and coarse end-inspiratory crepitations. These days, such end stage findings are rare and, in most current presentations, the examination will not reveal such occurrences.
A chest X-ray will be normal in half of cases. The best means of diagnosis is high-resolution CT scanning. CT scanning allows a direct measurement of the extent of the illness throughout the lung fields.
Sputum microbiology is useful in treating infections as they occur.
Measurement of inflammatory markers can give an indication of severity of the inflammatory burden and so the need for, or the response to treatment.
Investigations searching for a specific aetiology depend on the patient’s age and the stage of the disease.
If there is an underlying cause, such as immunoglobulin deficiency, it will need treatment. If the disease is well localised to a single lobe and is the result of a specific obstruction, then the thoracic surgeon may be able to cure the illness by resecting the affected area.
Postural drainage, or other sputum clearance techniques, carried out with the help of a physiotherapist or trained family helper can help to control sputum production.
Mucolytic drugs may be tried empirically, but the current evidence base available for such treatments is weak.
Antimicrobial chemotherapy is the mainstay of treatment. High doses are needed to penetrate the thickened and scarred bronchial walls to allow at least some of the antibiotic to penetrate into the infected mucus. The choice of antibiotic is best guided by knowledge of the infective organism in question.
To avoid high oral doses, nebulised or IV antibiotic administration can be useful.
In a considerable number of cases, chronic suppressive treatments may be needed to reduce the frequency of exacerbations.
This strategy is particularly common in CF patients. Patients with bronchiectasis may have either a restrictive or obstructive picture on lung function testing. It is also worth assessing patients for their response to bronchodilators such as salbutamol or ipratropium bromide.
Often, there is either a co-existent or consequent element of airways obstruction with bronchiectasis. This can be treated as it would be in asthma.
Use of oral steroids is justified in severe exacerbations. If there is good evidence of therapeutic response then inhaled corticosteroids can be continued, although evidence for this is not fully established. If there is documented improvement with these agents then it is justified to continue them. The decision is made on a patient by patient basis.
Lung transplantation is reserved for end-stage severe bronchiectasis, provided that the underlying disease is stable and not likely to jeopardise the transplanted lung.
Infective exacerbations are the most common complications, and may require admission to hospital. Chest pain and haemoptysis are common.
Empyema (pleural abscess) is seen much less often now that we have access to more effective antibiotics. Amyloidosis, arthropathy and vasculitis may occur in some rare cases as further complications of bronchiectasis.
Prognosis can be variable. Patients with more localised disease and fewer infective exacerbations will do better.
Chronic colonisation by Pseudomonas aeruginosa is a bad sign, but is ameliorated to some extent by aggressive antibiotic therapy. The pattern and prognosis of bronchiectasis has altered significantly over the last 100 years.
A Finnish study (published 1997) of hospitalised patients after discharge (1982-1986) followed up over ten years showed that about 13 per cent of patients with bronchiectasis died from their condition.
There are many patients with bronchiectasis that who live with their condition for years. However, towards the end of their life exacerbations become severe and more frequent.
Dr Davies is a GP in Illingworth, Halifax