Clinical Review - Bronchiectasis

Contributed by Dr Ailsa Sita-Lumsden, ST2 in respiratory medicine and Dr Robert Wilson, director of respiratory medicine and associate medical director, Royal Brompton Hospital, London

High resolution scan demonstrating severe bronchiectasis with dilated bronchi and thickened walls and characteristic signet rings
High resolution scan demonstrating severe bronchiectasis with dilated bronchi and thickened walls and characteristic signet rings

Section 1 - Epidemiology and aetiology
Bronchiectasis is a chronic disease resulting from the abnormal and permanent distortion of one or more of the bronchi.

The airways become abnormally dilated due to destruction of the elastic and muscle layers and become a collecting point for secretions, which are produced in excess by goblet cell hyperplasia and hypertrophy of the submucosal glands.

The epithelium is damaged, leading to loss of ciliated cells, which impairs clearance of these secretions.

Stationary mucus acts as a breeding ground for bacteria and is a source of recurrent and chronic bacterial infections.

These lead to further inflammation, which in turn causes further damage to the bronchial walls in a vicious cycle.

There are four types of bronchiectasis, varying in severity.

Cystic (saccular) is a severe form of bronchiectasis characterised by very large volumes of sputum production and finger clubbing. This is far less common now in the developed world.

Cylindrical is the most common type that runs a milder course. It commonly affects the lower lobes bilaterally.

In varicose bronchiectasis, bronchial walls have a beaded appearance due to scarring causing constriction superimposed on dilation.

Traction bronchiectasis is found in fibrotic lung diseases such as sarcoidosis and fibrosing alveolitis as airways are pulled apart by the fibrotic process.

Prevalence
According to 2005/2006 DoH statistics, one in 1,800 UK hospital admissions is due to bronchiectasis as the primary condition and bronchiestasis patients occupy approximately one in every 1,000 hospital bed days.1

Aetiology
In up to 50 per cent of cases no underlying cause is identified. However, there are some known causes of bronchiectasis (see box) and several other conditions have been associated with bronchiestatasis without a clear understanding of the aetiology.2

Causes of bronchiectasis

Infection: Severe bacterial respiratory tract infections*

Congenital: Deficiency of structural elements of the bronchial wall (e.g. Marfan's, Ehlers-Danlos syndromes)

Mechanical obstruction: Obstruction* (by a foreign body - most common in children, tumor, or stenosis) in association with recurrent infection

Immune*: e.g. hypogamma-globulinemia, common variable immunodeficiency, HIV

Inflammatory: Inhalation of corrosive gas or aspiration of gastric contents into the lungs*

Abnormal mucus clearance Primary ciliary dyskinesia, CF, Young's syndrome

Excessive immune response Allergic bronchopulmonary aspergillosis* or lung transplant rejection*

*Conditions or causes that have potential treatments

Section 2 - Diagnosis
Bronchiectasis is characterised by chronic sputum production with susceptibility to lower respiratory tract infections. These may be bacterial, viral or both, or there may be chronic bacterial infection causing daily purulent sputum production.

Exacerbations are usually associated with an increase in volume of sputum and purulence, although sputum production can also decrease as it becomes more viscous and difficult to clear.

Patients become short of breath with exertion, and chest discomfort may be present.Many patients have associated symptoms of rhinosinusitis.

There may be coarse crackles heard over the affected area. Wheezes occur secondary to airflow obstruction, which is largely irreversible, although some patients do have degrees of reversibility. There may also be squeaks, which are indicative of small airways disease.

Investigations
The diagnosis is confirmed on CT scanning. The defining characteristic is bronchial dilation with the internal diameter of the bronchial lumen greater than that of the adjacent artery.

Although chest X-rays are an insensitive screening test for bronchiectasis, they are useful for ruling out other pulmonary conditions.

Patients who are young, or those with deteriorating symptoms, should be investigated to determine the aetiology and severity of their condition.

Sputum analysis for colour and volume, measured over a 24-hour period, can be helpful in determining stable disease against an exacerbation.

Lung function is useful for monitoring disease severity, as lower FEV1 correlates with extent of bronchiectasis, and because it can be repeated easily can be used to monitor stability.

Advanced airflow obstruction may be seen in severe bronchiectasis, but there may also be restrictive or normal patterns.

Severe cases may develop right heart failure, which is a bad prognostic feature. Echocardiography should therefore be performed when concerned about cardiac involvement.

Aspergillus allergy and non-TB mycobacterial infection can both cause bronchiectasis, but also complicate pre-existing disease leading to deterioration in a previously stable case.

All problematic patients should be thoroughly investigated to identify any treatable cause of bronchiectasis. This will often require referral to a chest specialist.

Key Features in the History and Examination
Ask about:
  • Cough
  • Volume and colour of sputum production
  • Haemoptysis
  • Energy levels/fatigue
  • URTI: blockage, running, postnasal drip, facial pain
  • Chest pain
  • Frequency of exacerbations
  • Shortness of breath
Features of an exacerbation
  • Increased breathlessness
  • Increased sputum production
  • Sputum purulence
  • Pyrexia, often low grade
  • Pleuritic chest pain - this is a serious complication as it restricts the cough
  • Minor haemoptysis (major haemoptysis is rare)
Key features in examination
  • Crackles over affected areas
  • Obstructive spirometry, often with little reversibility
  • Clubbing is rarely present because cystic bronchiectasis is now rare due to improvements in treatment
  • The nose may be obstructed with evidence of inflammation

 

Investigations of Bronchiectasis

Patients in the community

  • Blood investigations: FBC with white cell differential; CRP; routine biochemistry; aspergillus IgG and RAST; rheumatoid factor and other auto-antibodies if clinically indicated; protein electrophoresis strip; alpha1-antitrypsin
  • Chest radiograph - PA and lateral
  • Spirometry
  • Sputum culture and sensitivity
  • Sputum smear and culture for acid-fast bacilli

 

Section 3 - Management
Stable disease

The aim of management is to reduce symptoms, limit exacerbations, preserve lung function and improve quality of life.

The mainstays of treatment are daily physiotherapy to drain the affected areas and appropriate antibiotics to treat infection and reduce inflammation.

Physiotherapy is an important element of the treatment, aiming to improve the efficiency of clearing sputum.

All patients should be reviewed by a specialist chest physiotherapist for training in active cycle breathing technique and assisted devices (e.g. the Flutter mucus clearing device) that have similar outcomes to traditional postural drainage but are easier for the patients to master.3

Bronchodilators
There is a role for bronchodilator therapy and inhaled hyperosmolar agents, such as nebulised hypertonic 7% saline, as adjuncts to physiotherapy. The inhaled beta-2 agonists and/or anticholinergics may be used prior to chest physiotherapy to minimise bronchial hyper-reactivity, and may also help to relieve breathlessness.

Use of high-dose inhaled corticosteroids (fluticasone 500 microgram twice daily or beclometasone 750 microgram twice daily) have shown a reduction in 24-hour sputum volume and improvement in quality of life but no impact on FEV1 or exacerbation frequency.4 Their routine use in bronchiectasis should be discouraged.

Antibiotic therapy
Long-term continuous antibiotic therapy, which can be given as oral, nebulised or planned IV courses, may be indicated in patients having frequent exacerbations that are impacting on quality of life.

These patients have chronic infection, often with pseudomonas species, and it is thought that the antibiotics act to reduce airway inflammation by decreasing the bacterial population of the airways. There is a definite risk that antibiotic prophylaxis will encourage resistance of the lung flora, therefore it must be limited to patients who, despite optimum medical management, continue to have frequent exacerbations.

More recently there has been interest in the anti-inflammatory role of some antibiotics, particularly the macrolides - a promising area of research.

Surgery may be an option if the bronchiectasis is localised to one area of the lung, and should be considered if medical management has not controlled symptoms, and lung function is adequate. Because most cases of tubular bronchiectasis are bilateral, surgery is unsuitable.

Exacerbations
Prompt empirical antibiotic therapy should be commenced, based on previous sputum microbiology (if available). Fresh sputum cultures should be sent for microbiological culture.

Treatment should be adjusted if there is no clinical improvement and should be guided by culture and sensitivity results. Antibiotics should be continued for 10-14 days.

Increased chest physiotherapy at times of exacerbation will help to clear secretions and the patient should be advised to keep well hydrated. Patients may also require a short course of oral prednisolone in severe exacerbations and/or modification of their bronchodilator therapy.

Antibiotics commonly used in bronchiectasis
Non-pseudomonas
NameDosage and duration  Comment
Co-amoxiclav625mg three times daily 10-14 daysMany Haemophilus influenzae, and most Morexella catarrhalis,
produce beta-lactamase so are
resistant to amoxicillin
Doxycycline200mg once daily first day, 100mg once daily 9-13 days
100mg twice a day in severe case
once daily Warn patient of photosensitivity
Cefpodoxime200mg twice a day 10-14 daysBest oral cephalosporin against Haemophilus influenzae; 400mg
twice a day in severe cases
Azithromycin500mg once daily six days
Treat with a further 250mg once daily for six days in severe cases
Pseudomonas
Ciprofloxacin750mg twice a day 10-14 days500mg twice a day if higher dose not tolerated
Azithromycin500mg once daily six days
Treat with a further 250mg once in severe cases; does not kill pseudomonas but reduces pathogenicity and inflammation

Section 4 - Prognosis

Prognosis varies widely, depending on how widespread and severe the bronchiectasis, and the presence of other underlying disorders.

Most patients with bronchiectasis have a reasonable outlook. Treatment, in particular antibiotic treatment for any infection, or regularly when needed, keeps most patients reasonably well.

Patients with co-existing conditions, such as emphysema and immunoglobulin deficiency, and patients who have complications, such as pulmonary hypertension or cor pulmonale, tend to have a worse prognosis.

A life-threatening bleed from a damaged airway may also occur, but is rare.

When to refer

The majority of patients can be managed in general practice. However, it is important that young patients are referred for investigations into possible underlying causes.

It is also appropriate to refer patients whose symptoms are unstable or progressing.

The last group to consider for referral are those patients with associated conditions because they would benefit from specialist opinion.

Resources

References

1. Department of Health website http://www.hesonline.nhs.uk

2. Pasteur M, Helliwell S, Houghton S et al. An investigation into causative factors in patients with bronchiectasis. Am J Respir Crit Care Med 2000; 162(4 Pt 1): 1,277-84.

3. Thompson C, Harrison S, Ashley J, Day K, Smith D. Randomised crossover study of the Flutter device and the active cycle of breathing technique in non-cystic fibrosis bronchiectasis. Thorax 2002; 57: 446-8.

4. Tsang K, Tan K, Ho P et al. Inhaled fluticasone in bronchiectasis: a 12-month study. Thorax 2005; 60: 239-43.

Further reading

- Wilson R. 'Bronchiectasis'. In: Gibson GJ, Geddes DM, Costabel U, Sterk PJ and Corrin B (eds.) Respiratory Medicine, 2003, pp. 1,445-64. London: Saunders/Elsevier.

Patient support

www.bronchiectasis.info A support forum for patients with bronchiectasis

- For an archive of all GP clinical reviews visit www.healthcarerepublic.com/clinical/GP

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