Bronchiectasis: a GP guide

Respiratory specialist Dr Anthony De Soyza describes the classic symptoms, differential diagnosis and management of bronchiectasis

Bronchiectasis is a disease state defined by localised, irreversible dilation of part of the bronchial tree. It is classified as an obstructive lung disease. Involved bronchi are dilated, inflamed and easily collapsible, resulting in airflow obstruction and impaired clearance of secretions.

Signs and symptoms

Bronchiectasis is defined by the presence of one or more abnormally and permanently dilated bronchi. It should be viewed as a common end-stage condition caused by a huge variety of diseases. It has a wide severity spectrum of clinical symptoms that are not always related to the extent of the underlying bronchial abnormalities, ranging from an asymptomatic diagnosis noted on an incidental chest X-ray or CT scan, to the more classical presentation of:

• persistent cough with copious mucopurulent sputum and intermittent haemoptysis

• shortness of breath and impaired exercise tolerance

• fatigue

• frequent infective exacerbations requiring antibiotics.

Differential diagnosis

Bronchiectasis is under-diagnosed and often diagnosed late. It should be considered in patients with both asthma and COPD when mucus production or recurrent infections are predominant symptoms.

Notably, asthma, COPD and bronchiectasis are all associated with airflow obstruction on spirometry so there is no single definitive test. Where the clinical course does not seem typical for asthma or COPD, then referral or CT scanning should be considered. Other diseases to consider that may present in a similar way include:

• upper-airway cough syndrome (such as postnasal drip syndrome or gastro-oesophageal reflux disease)

• lung malignancy

• TB

• pneumonia

• lung abscess.

Any chronic cough should be investigated further with at least a plain radiograph to exclude a mass lesion. Further tests or referral should be considered thereafter.

Less common are immune deficiencies – including common variable immunodeficiency as well as acquired hypogammaglobulinaemia secondary to HIV infection – chemotherapy, nephrotic syndrome and haematological malignancies. Allergic bronchopulmonary aspergillosis (ABPA) can occur in asthmatics as a result of an excessive immune response to inhaled Aspergillus fumigatus and may in time lead to bronchiectasis. Rarer causes include diseases of mucociliary clearance such as primary ciliary dyskinesia, Kartagener's syndrome and Young's syndrome.

Investigations and imaging

Suggested investigations in primary care are chest X-ray, spirometry, sputum analysis for bacteria, mycobacteria and fungi, and FBC.

• Chest X-ray is commonly normal in bronchiectasis, but may exclude other differential diagnoses.

• Basic spirometry is likely to be normal or show an obstructive pattern (a reduced FEV1/FVC ratio).

• FBC is often normal but may show anaemia, polycythaemia, neutrophilia or eosinophilia.

In secondary care, a high-resolution CT scan may be carried out to confirm and characterise the disease. Additional specific tests will be guided by the suspected underlying cause. Testing for cystic fibrosis is required in selected cases.

Treating acute exacerbations

The most common organisms in exacerbations are similar to those that cause lower respiratory tract infections and community-acquired pneumonia, including Haemophilus influenzae, Streptococcus pneumoniae, Moraxella catarrhalis and Staphylococcus aureus. Up to 20% of cases may have Pseudomonas aeruginosa.

Recently there has been a move towards patients with pseudomonas infection having early aggressive therapy in an attempt to eradicate this infection; most specialists would want such patients referred for assessment. Candida species are felt non-pathogenic in general, while aspergillus fungal infection should prompt repeated testing and consideration for referral.

Where possible, sputum should be sent for culture and sensitivity testing before starting antibiotics. In the absence of previous sputum results, monotherapy with amoxicillin or doxycycline would be good choices, but should be adjusted in light of subsequent sputum sensitivities.

Negative sputum cultures do not exclude an infective exacerbation. There is inadequate evidence to guide optimum antibiotic duration, but current consensus suggests the use of a higher dose of antibiotics for a longer period of time.

Maintenance therapy

Long-term nebulised anti-pseudomonal antibiotics with colomycin, gentamicin or tobramycin as maintenance therapy are used at specialist centres and may be considered on an individual patient basis. Generally persistent Pseudomonas, S. aureus and Enterobacter species with recurrent exacerbations will prompt consideration of nebulised antibiotics.

Non-pharmacological treatment

The two main management principles in bronchiectasis are:

• maintaining bronchopulmonary hygiene using methods to enhance mucociliary clearance, and

• preventing and treating infective exacerbations.

The overall aims are to improve daily symptoms and reduce exacerbation rates, as well as prevent complications and decline in lung function.

In patients with hypersecretion of mucus, specialist chest physiotherapists should be involved to tailor sputum clearance techniques.

Most frequently used methods include the ‘active cycle of breathing technique' or airway oscillation with a Flutter or Acapella device. An expert physio should assess a patient and advise on the most suitable method for the patient and their lifestyle.

Surgical options and new therapies

In a selected group of patients with focal bronchiectasis that is unresponsive despite maximal medical therapy, surgical intervention may be considered.

Bilateral lung transplantation is occasionally considered in those with end-stage lung disease. Surgery should be seen as a last resort, particularly as a number of new therapies are emerging.

New therapies include targeting airway inflammation, for example using a chronic macrolide antibiotic at sub-microbicidal doses, or targeting inflammatory cell biology.

Chronic macrolide therapy should not be started until mycobacterial infection is excluded and is generally felt best monitored by specialists.

There are also trials of nebulised or inhaled maintenance antibiotics and new mucolytics such as nebulised hypertonic saline or osmotic agents such as mannitol.

It is generally believed that mucus retention, airway inflammation and infections form a triad of targets for treatment. Treating one aspect of this triad in isolation may be insufficient to maintain stability.

Complications to look out for

Although bronchiectasis is a disease that primarily affects the lungs, there are many associated extrapulmonary manifestations. These include:

• rhinosinusitis

• fatigue

• urinary incontinence

• osteoporosis

• arthropathy.

These complications should be treated in their own right. Urinary incontinence is very rarely spontaneously reported, yet can affect even young female patients.

Many patients with bronchiectasis have an expectation that recurrent exacerbations are normal. A number of therapeutic options should be trialled prior to accepting more than two to three exacerbations per year.

Pneumonia and influenza are probably over-represented in this group and certainly are likely to be poorly tolerated, so immunisation should be strongly considered.

As yet, no haemophilus vaccine relevant to the non-typeable haemophilus infections seen in this patient group has emerged. The HiB vaccine has no proven role in this patient group and haemophilus type B infection is felt to be rare.

Dr Anthony De Soyza is senior lecturer at the institute of cellular medicine at Newcastle University and honorary consultant in the bronchiectasis service at the Freeman Hospital, Newcastle upon Tyne Hospitals

Competing interests None declared