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Non-cystic fibrosis bronchiectasis 

Non-cystic fibrosis bronchiectasis
Chapter:
Non-cystic fibrosis bronchiectasis
Author(s):

Jeremy Hull

, Julian Forton

, and Anne Thomson

DOI:
10.1093/med/9780199687060.003.0033
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Introduction

Bronchiectasis is defined as a dilatation of the airway, accompanied by an inflammatory destruction of the bronchial and peribronchial tissue. It was previously thought to be irreversible, but there is now clear evidence that early bronchiectasis in children can resolve. The pathogenesis is thought to be the breakdown of local lung defences, leading to infection, inflammation, bronchial obstruction, airway damage, ineffective mucociliary clearance, and persistent retention of secretions and infection.

Prevalence

There have been a number of recent attempts at estimating prevalence.

  • North-east England: 172/million children aged <15 years.

  • New Zealand: 335/million children aged <15 years.

  • Aboriginal population of Alice Springs, Australia: 10 000/million.

Aetiology

Careful investigation can reveal an underlying aetiology in 60–70% of cases. In most populations, a post-infectious cause is most common, followed by immune deficiency (Box 33.1).

Clinical features

The presenting clinical feature is a chronic productive cough, with or without:

  • purulent sputum (young children rarely expectorate);

  • chest pain during exacerbation;

  • wheezing;

  • breathlessnes on exertion;

  • haemoptysis;

  • recurrent LRTIs.

Physical examination may be normal or may reveal:

  • clubbing;

  • inspiratory crepitations.

A chronic productive-sounding cough that persists or recurs after initial treatment with physiotherapy and antibiotics warrants further investigation (see Non-cystic fibrosis bronchiectasis Chapter 4).

Investigations and diagnosis

Imaging

  • CXR. May show evidence of bronchial wall thickening or airway dilatation and/or areas of volume loss. However, it may be normal.

  • HRCT. Required for diagnosis. Features of bronchiectasis are:

    • bronchial wall thickening;

    • the internal diameter of the bronchus cut in cross-section being larger than that of the accompanying artery (the signet ring sign);

    • poorly tapering bronchi as they move to the periphery of the lung;

    • bronchi clearly seen near the periphery of the lung.

  • Bronchiectasis occurs in lower lobes > middle lobe > upper lobes.

  • Bilateral upper lobe bronchiectasis is more common in CF than in other causes of bronchiectasis. Unilateral upper lobe bronchiectasis can be seen after TB. Focal bronchiectasis, particularly of the lower lobes, makes foreign body more likely. Multiple lobe involvement can be seen after viral pneumonia, aspiration (particularly bilateral lower lobe disease), or associated with a systemic disorder such as immune deficiency or PCD.

Bronchoscopy

Bronchoscopy is not always required, e.g. in children with known immune deficiency or PCD, bronchoscopy will add little (unless samples are needed for microscopy or culture).

Thresholds for carrying out bronchoscopy vary in different centres. We suggest that bronchoscopy should be carried out:

  • when there is focal bronchiectasis (to exclude airway narrowing);

  • when the HRCT suggests the possibility of an airway abnormality;

  • to assist in the investigation of possible aspiration lung disease.

Other investigations looking for an aetiology

  • Sweat test—always worth repeating.

  • CFTR genetic mutation analysis may be helpful, if CF is suspected but the sweat test is borderline or normal.

  • FBC for lymphocyte and neutrophil counts.

  • Ig levels, including IgE and IgG subclasses. Low levels of subclasses of IgG are especially important if IgA levels are also low (see Non-cystic fibrosis bronchiectasis Chapter 35).

  • Specific antibody levels to vaccinations and the response to booster doses if the initial response is poor.

  • More detailed immunological tests should be considered where there is a clinical suspicion of an immunological abnormality and the initial tests are normal.

  • Ciliary brush biopsy of the nose or trachea (if bronchoscopy performed).

  • Contrast swallow and pH study (evidence of GOR makes aspiration more likely).

  • Videofluoroscopy if aspiration is suspected.

  • HIV test, if high risk or other evidence of immune deficiency.

Microbiology

Airway culture may suggest an aetiology, e.g. the presence of Pseudomonas species makes CF likely. Close attention to airway infection is an essential part of the management of bronchiectasis to limit any progressive damage. Samples can be obtained by:

  • sputum, including induced sputum;

  • cough swab;

  • BAL.

Lung function testing

Many children with mild bronchiectasis will have normal lung function. In more severe disease, airways obstruction and a degree of restriction can occur.

Management

The overall aim of treatment is to control symptoms, prevent progressive lung disease, and ensure normal growth and development. Where a cause of the bronchiectasis is found, specific management will be directed to the cause, e.g. surgery to prevent aspiration, replacement of Ig for deficiency. Day-to-day treatment is a combination of airway clearance and antibiotic use. Clearing secretions from the dilated bronchial tree and preventing further infection can halt disease progression and, in early cases, provide an environment for healing.

Physiotherapy

There are no trials of physiotherapy in this condition, but plenty of anecdotal evidence that it is beneficial. Children should be seen by a physiotherapist at regular clinic appointments and encouraged to use the airway clearance technique which works best for them.

Antibiotics

The most commonly found organisms are:

  • non-encapsulated Haemophilus influenzae;

  • Streptococcus pneumoniae;

  • Moraxella catarrhalis.

Some children respond to antibiotics, such as amoxicillin, given only at times of exacerbation and, between exacerbations, produce only small amounts of clear secretions. Other children benefit from continuous antibiotic such as cefixime.

Infection with Pseudomonas aeruginosa does sometimes occur in children with non-CF bronchiectasis, and both treatment and eradication regimes similar to those used in CF have been used, although there is no evidence that they are beneficial. In a trial in adults with non-CF bronchiectasis, the addition of regular nebulized antibiotics did not affect the clinical outcome.

Other agents

  • Dornase alfa was not helpful in adult trials of non-CF bronchiectasis1 and probably is not helpful in children with this condition.

  • Bronchodilators may be helpful in children where wheeze is a prominent feature.

  • Two studies of inhaled steroids in adults with bronchiectasis have shown a decrease in the 24 h sputum volume, exacerbation frequency, and dyspnoea, and an improvement in quality of life scores,2,3but routine use is not recommended outside of those patients with additional asthma symptoms.

  • Azithromycin. Pilot and non-controlled studies have suggested that the regular use of azithromycin decreases exacerbations in adults.

Surgery

Surgery is an option for localized bronchiectasis if:

  • there are significant symptoms not responding to medical treatment;

  • there is resectable disease causing persistent focal infection;

  • there is life-threatening haemoptysis.

Outcome

Children with bronchiectasis should be followed regularly in the outpatient clinic, with monitoring of their symptoms, growth, and lung function. It may be necessary to repeat the HRCT to determine if there is disease progression.

The long-term outcome is dependent on the underlying condition. In most cases, treatment should halt progression. There are well-recorded cases of improvement and reversal of bronchiectasis in children.4

Further information

Fall A, Spencer D (2006). Paediatric bronchiectasis in Europe: what now and where next? Paediatr Respir Rev 7, 268–74.Find this resource:

Pasteur MC, Bilton D, Hill AT; British Thoracic Society Bronchiectasis non-CF Guideline Group (2010). British Thoracic Society guideline for non-CF bronchiectasis. Thorax 65 (Suppl. 1), i1–58.Find this resource:

Notes:

1 O’Donnell AE, Barker AF, Ilowite JS, Fick RB (1998). Treatment of idiopathic bronchiectasis with aerosolized recombinant human DNase I. rhDNase Study Group. Chest 113, 1329–34.

2 Martinez-Garcia MA, Perpina-Tordera M, Roman-Sanchez P, Soler-Cataluna JJ (2006). Inhaled steroids improve quality of life in patients with steady-state bronchiectasis. Respir Med 100, 1623–32.

3 Tsang KW, Tan KC, Ho PL, et al. (2005). Inhaled fluticasone in bronchiectasis: a 12 month study. Thorax 60, 239–43.

4 Gaillard EA, Carty H, Heaf D, Smyth RL (2003). Reversible bronchial dilatation in children: comparison of serial high-resolution computer tomography scans of the lungs. Eur J Radiol 47, 215–20.