Bronchiectasis

oPatientPlus articles are written by UK doctors and are based on research evidence, UK and European Guidelines. They are designed for health professionals to use, so you may find the language more technical than the condition leaflets.

Bronchiectasis is a persistent or progressive condition characterised by dilated thick-walled bronchi.[1] It may be widespread throughout the lungs or more localised. It may develop after lung infections, particularly in childhood and in association with underlying problems, eg immunodeficiency, cystic fibrosis. The clinical course of bronchiectasis is characterised by chronic cough with purulent sputum associated with acute exacerbations.[2]

Bronchiectasis can be classified into the following forms (all 3 forms may be present in the same patient):

  • Cylindrical bronchiectasis: bronchi are enlarged and cylindrical.
  • Varicose bronchiectasis: bronchi are irregular with areas of dilatation and constriction.
  • Saccular or cystic: dilated bronchi form clusters of cysts. This is the most severe form of bronchiectasis and is often found in patients with cystic fibrosis.

The affected airways are inflamed and easily collapse. There is an impairment of airflow and drainage of secretions, leading to the accumulation of a large amount of mucus in the lungs. The mucus collects bacteria, predisposing to frequent and often severe lower respiratory tract infections. The severity of bronchiectasis used to be classified according to the volume of sputum produced but this has now largely been superseded by using the radiological appearance on CT scan.

  • The incidence varies between populations from 3.7/100,000 children in New Zealand to 52/100 000 adults in the USA. Features of bronchiectasis found in chest X-rays (CXRs) in the UK in the 1950s suggested a prevalence of 100/100,000.[1]
  • The incidence has decreased as a result of immunisation programmes and more effective antibiotics.[3]
  • Bronchiectasis can present at any age but increases with age and the highest prevalence is in older women.[4]
  • Women tend to be more severely affected than men.[5]

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The disease is caused by infection but there are certain conditions that facilitate infections, which therefore tend to be recurrent and more severe and so cause damage to the lungs. However, there is no identifiable underlying cause in about 50% of adults and 25% of children.[6][7]

Smoking does not appear to be an independent risk factor but smoking cessation is important if the patient does smoke.

  • The symptoms vary from intermittent episodes of expectoration and infection localised to the region of the affected lung to persistent daily expectoration of large volumes of purulent sputum.
  • There may be associated nonspecific respiratory symptoms including dyspnoea, chest pain and haemoptysis. Bronchiectasis may progress to respiratory failure and cor pulmonale.
  • All causes of bronchiectasis should be considered and assessed as appropriate.
  • All patients with bronchiectasis should be assessed for upper respiratory tract symptoms.

Children

  • Respiratory symptoms, especially cough and sputum production, should be assessed and recorded in all children with possible bronchiectasis.
  • There should be a high index of suspicion for diagnosing bronchiectasis in children with chronic respiratory symptoms.
  • Persistent lung crackles on auscultation indicate possible underlying bronchiectasis.
  • Children should be assessed for bronchiectasis if they present with:
    • Chronic moist/productive cough, especially between viral colds or with positive bacterial cultures.
    • Asthma that does not respond to treatment.
    • A child with chronic respiratory symptoms and a single positive sputum culture for Staphylococcus aureus, Haemophilus influenzae, Pseudomonas aeruginosa, non-tuberculous mycobacteria or Burkholderia cepacia complex.
    • An episode of severe pneumonia, especially if there is incomplete resolution of symptoms, physical signs or radiological changes.
    • Pertussis-like illness failing to resolve after 6 months.
    • Recurrent pneumonia.
    • Persistent and unexplained physical signs or abnormalities on CXR.
    • Localised chronic bronchial obstruction.
    • Respiratory symptoms in children with structural or functional disorders of the oesophagus and upper respiratory tract.
    • Unexplained haemoptysis.
    • Respiratory symptoms with any clinical features of cystic fibrosis, primary ciliary dyskinesia or immunodeficiency.

Adult

  • Assessment of symptoms in patients with bronchiectasis should include a record of both sputum purulence and estimated or measured 24-hour sputum volume when clinically stable.
  • The number of infective exacerbations per annum should be noted including frequency and nature of antibiotic usage.
  • Adults should be assessed for bronchiectasis if they present with:
    • Persistent productive cough, especially if any one of the following:
      • Young age at presentation.
      • History of symptoms over many years.
      • Absence of smoking history.
      • Daily expectoration of large volumes of very purulent sputum.
      • Haemoptysis.
      • Sputum colonisation with P. aeruginosa.
    • Unexplained haemoptysis or nonproductive cough.
    • Patients thought to have chronic obstructive pulmonary disease (COPD) may have bronchiectasis alone or as well as COPD and referral for investigation is appropriate if:
      • Management is not straightforward.
      • There is slow recovery from lower respiratory tract infections.
      • There are recurrent exacerbations.
      • There is no history of smoking.

Investigations are directed at identifying any underlying cause and to establish the severity of disease.

  • Routine blood tests are nonspecific but may confirm the presence of infection or presence of any comorbidity. FBC may show raised white cell count (infection) or polycythaemia (in advanced cases).
  • Immune function tests:
  • The following should be measured in all patients:
    • Serum immunoglobulins (IgG, IgA, IgM) and serum electrophoresis.
    • Serum IgE, skin prick testing or serum IgE testing to Aspergillus fumigatus and aspergillus precipitins.
    • All patients with bronchiectasis should be screened at presentation for gross antibody deficiency by routine measurement of serum IgG, IgA and IgM levels and serum electrophoresis.
    • Respiratory and immunology units should develop additional local protocols for screening assessment of humoral responses to specific antigens.
    • Measurement of baseline specific antibody levels against tetanus toxoid and the capsular polysaccharides of both Streptococcus pneumoniae and H. influenzae type b (or suitable alternative peptide and polysaccharide antigens).
    • Immunisation with appropriate vaccines followed by re-assay of individual specific antibody responses after 21 days where screening baseline levels are low.
  • Further assessment of immune competence is recommended in the following circumstances:
    • Antibody screening investigations have demonstrated the presence of an antibody deficiency disorder.
    • In the presence of normal antibody screening test results where the following are present:
      • Clinical suspicion of immune deficiency (short stature, facial abnormality, cardiac lesions, hypocalcaemia, cleft palate, oculocutaneous telangiectasia, eczema, dermatitis, petechiae, manifestations of endocrinopathy, unexplained failure to thrive, enlargement or absence of lymphoid tissues, unexplained organomegaly, unexplained joint symptoms).
      • A family history of known or suspected immune deficiency.
      • Infections which are serious, involving a threat to life, tissue destruction or which require/have required surgical intervention (eg lobectomy, tonsillectomy, insertion of grommets, incision of boils), are persistent or recurrent despite multiple or prolonged courses of antibiotics, involve unusual/opportunistic micro-organisms or involve multiple sites (eg sinuses or middle ear in addition to the bronchial tree).
  • Gastrointestinal investigations:
    • There should be a low threshold for gastrointestinal investigations in children.
    • Gastric aspiration should be considered in patients following lung transplantation.
  • Cystic fibrosis:
    • All children and all adults up to the age of 40, presenting with bronchiectasis, should have investigations for cystic fibrosis.
    • In adults, investigations should also be considered in those with:
      • Age at presentation >40 years and no other identified cause.
      • Persistent isolation of S. aureus in the sputum.
      • Features of malabsorption.
      • Male primary infertility.
      • Upper lobe bronchiectasis.
      • A history of childhood steatorrhoea.
    • Screening investigations should include both 2 measurements of sweat chloride, and cystic fibrosis transmembrane regulator (CFTR) genetic mutation analysis.
  • Tests for ciliary function:
    • Ciliary investigations should be considered in children with bronchiectasis when there is:
      • No other cause for bronchiectasis identified.
      • A history of continuous rhinitis since the neonatal period.
      • A history of neonatal respiratory distress.
      • Dextrocardia.
    • Ciliary investigations should be considered in adults only if there is a history of chronic upper respiratory tract problems or otitis media. Factors favouring investigation include:
      • Problems since childhood.
      • Childhood chronic otitis media.
      • Predominantly middle lobe bronchiectasis.
      • Infertility or dextrocardia.
    • For adults, the saccharin test and/or exhaled nasal nitric oxide (NO) may be used to screen out those not requiring detailed ciliary function tests.
  • Radiological investigations:
    • CXR: a baseline CXR should be done in all patients. Repeat CXRs need only be done if clinically indicated. CXR may be normal or show ring or tubular opacities, tramlines and fluid levels.
    • The gold standard for diagnosis is high-resolution CT (HRCT) of the chest.[8] HRCT has a very high sensitivity and specificity for diagnosis and has now replaced bronchography.[9][10]
    • HRCT features of bronchiectasis: bronchial wall dilation (internal lumen diameter greater than accompanying pulmonary artery or lack of tapering) is the characteristic feature; bronchial wall thickening is often also present although harder to define.
    • The severity of bronchiectasis on HRCT correlates with measures of airflow obstruction.
    • Routine repeat CXR or HRCT is not necessary; repeat imaging should be considered when there is clinical need. In cases of humoral immune deficiency, repeating HRCT at intervals may be necessary to detect asymptomatic progression.
  • Sputum microbiology:
    • All children and adults with bronchiectasis should have an assessment of lower respiratory tract microbiology.
    • Respiratory tract specimens should be obtained in all patients with bronchiectasis. To maximise the chances of isolating H. influenzae and S. pneumoniae, specimens should reach the microbiology laboratory within 3 hours.
    • Persistent isolation of S. aureus (and/or P. aeruginosa in children) should lead to consideration of underlying bronchopulmonary aspergillosis or cystic fibrosis.
  • Lung function tests:
    • In all children who are old enough (usually aged >5 years) forced expiratory volume in 1 second (FEV1), forced vital capacity (FVC) and forced expiratory flow (FEF) 25-75% should be measured at initial assessment.
    • All adults with bronchiectasis should have measures of FEV1, FVC and peak expiratory flow (PEF).
    • Repeat assessment of FEV1, FVC and PEF should be made at least annually in those patients attending secondary care.
    • Patients with immune deficiency or primary ciliary dyskinesia should have measurements of FEV1, and FVC at least four times each year.
    • Measurement of lung volumes and gas transfer coefficient may help in the identification of other causes of airflow obstruction such as COPD or emphysema.
    • Reversibility testing may identify improvement in lung function after bronchodilators and should always be considered if airflow obstruction is identified, especially in young people.
    • Exercise tests have a role in investigating children in whom symptoms are out of keeping with lung function or HRCT measurements. In adults, exercise testing should be part of a pulmonary rehabilitation programme.
    • FEV1 and FVC should be measured before and after intravenous antibiotic therapy as this may give objective evidence of improvement.
    • Spirometry and lung volumes should be measured in all patients before and after commencing long-term oral or nebulised antibiotic therapy.
  • Bronchoscopy:
    • In children, bronchoscopy is indicated when bronchiectasis affects a single lobe to exclude a foreign body. In some acutely ill patients it may achieve a useful microbiological result.
    • In adults with localised disease, bronchoscopy may be indicated to exclude proximal obstruction.
    • In adults, bronchoscopy and bronchoscopic sampling of the lower respiratory tract does not have a place in the routine investigation of patients with bronchiectasis.
    • For patients in whom serial testing of sputum does not yield microbiological information and who are not responding well to treatment, bronchoscopic sampling of lower respiratory tract secretions may be indicated.
    • Bronchoscopy is indicated if HRCT suggests atypical mycobacterial infection and sputum culture is negative.
    • Cytological examination of bronchoscopic specimens can provide evidence supporting gastric aspiration.

Damaged lung cannot be repaired and so the basis of management is to prevent or at least slow down further deterioration. Management includes treatment of any underlying cause and any coexisting medical problems. Patients who should have regular follow-up in secondary care include:[1]

  • All children with bronchiectasis
  • Patients with chronic P. aeruginosa, opportunist mycobacteria or meticillin-resistant S. aureus (MRSA) colonisation.
  • Deteriorating bronchiectasis with declining lung function.
  • Recurrent exacerbations (3 or more per year).
  • Patients receiving prophylactic antibiotic therapy (oral or nebulised).
  • Patients with bronchiectasis and associated rheumatoid arthritis, immune deficiency inflammatory bowel disease and primary ciliary dyskinesia.
  • Patients with bronchopulmonary aspergillosis.
  • Patients with advanced disease and those considering transplantation.

General management issues

  • Maintain a healthy diet and physical exercise; ensure adequate energy intake to provide for increased energy demands.
  • If the patient smokes this must be stopped and smoking cessation advice and help should be offered. Passive smoking should also be avoided
  • Immunisation against influenza and pneumococcus.[11][12]

Physiotherapy: airway clearance techniques and exercise

  • Patients should be made aware of the airway clearance techniques available and, where possible, be encouraged to be independent with the chosen airway clearance technique.
  • Modified gravity-assisted positions (no head-down tilt) should be offered where the conventional tipped position is contra-indicated or unacceptable to the patient.
  • During an acute exacerbation or when the patient is more fatigued than usual, manual techniques may be offered as a part of an airway clearance technique regimen.
  • Sterile water inhalation may be used before airway clearance to facilitate clearance. The use of nebulised normal or hypertonic saline prior to airway clearance could be considered to increase sputum yield, reduce sputum viscosity and improve ease of expectoration.
  • Inhaled beta-2 agonists should be used prior to treatment to enhance sputum clearance.
  • Noninvasive ventilation or intermittent positive pressure breathing may be used to augment tidal volume and reduce the work of breathing in those patients who are becoming fatigued and finding their standard airway clearance difficult.
  • Pulmonary rehabilitation should be offered to individuals who have breathlessness affecting their activities of daily living.

Drug treatment

  • Antibiotics:[1]
    • Before starting antibiotics, a sputum sample should be sent off for culture. Empirical antibiotics should be started while awaiting sputum microbiology.
    • If there is no previous bacteriology, first-line treatment is amoxicillin 500 mg three times a day or clarithromycin 500 mg twice daily (in patients who are penicillin-allergic) for 14 days.
    • High-dose oral regimens (eg amoxicillin 1 g three times a day or amoxicillin 3 g twice daily) may be needed in patients with severe bronchiectasis chronically colonised with H. influenzae.
    • Ciprofloxacin should be used in patients colonised with P. aeruginosa.
    • Sputum results, including previous sputum bacteriology results, are useful in deciding which antibiotic to use for acute exacerbations (all treatments are for 14 days):
      • S. pneumoniae: amoxicillin (second-line: clarithromycin)
      • H. influenzae (beta-lactamase negative): amoxicillin (second-line: clarithromycin or ciprofloxacin or ceftriaxone).
      • H. influenzae (b-lactamase positive): co-amoxiclav (second-line: clarithromycin or ciprofloxacin or ceftriaxone).
      • Moraxella catarrhalis: co-amoxiclav (second-line: ciprofloxacin).
      • S. aureus: flucloxacillin (second-line: clarithromycin).
      • Meticillin-resistant S. aureus (MRSA): oral rifampicin plus doxycycline/trimethoprim; intravenous vancomycin or teicoplanin.
      • Coliforms (eg Klebsiella spp., enterobacteria): oral ciprofloxacin (second line: ceftriaxone).
      • P. aeruginosa: oral ciprofloxacin (second-line: intravenous ceftazidime or tazocin or aztreonam or meropenem).
    • Antibiotics can be modified once the pathogen is isolated only if there is no clinical improvement and the treatment should then be guided by antibiotic sensitivity results.
    • Failure to respond to an antibiotic course should prompt a repeat sputum culture.
    • Intravenous antibiotics should be considered when patients are particularly unwell, have resistant organisms or have failed to respond to oral therapy (this is most likely to apply to patients with P. aeruginosa).
    • Long-term oral antibiotics:
      • Patients having 3 or more exacerbations per year requiring antibiotic therapy or patients with fewer exacerbations that are causing significant morbidity should be considered for long-term antibiotics.
        • S. pneumoniae: amoxicillin (second-line: clarithromycin).
        • H. influenzae (beta-lactamase negative): amoxicillin (second-line: clarithromycin).
        • H. influenzae (beta-lactamase positive): co-amoxiclav (second-line: clarithromycin).
        • M. catarrhalis: co-amoxiclav (second-line: clarithromycin).
        • MRSA: flucloxacillin (second-line: clarithromycin).
        • Children and adults chronically colonised with P. aeruginosa: nebulised gentamicin, tobramycin, or colistin.
      • In the first instance, high doses should not be used to minimise side-effects.
      • The antibiotic regimen should be determined by sputum microbiology when clinically stable. Long-term quinolones are currently not recommended. Macrolides may have disease-modifying activity but this has so far not been proven large randomised controlled trial.
      • Long-term antibiotics may result in antibiotic resistance in individual patients and alternative antibiotics should be chosen depending on sensitivity results.
      • Some patients may respond to antibiotic treatment despite resistance to that drug in vitro. Antibiotics should only be changed if there is no clinical response.
  • Other drug treatment:
    • Mucolytics and hyperosmolar agents: recombinant human DNase should not be used in adults or children with bronchiectasis.
    • Bronchodilators: it is appropriate to assess patients with airflow obstruction for reversibility to beta-2 agonist and anticholinergic bronchodilators and to start treatment if lung function or symptoms improve on therapy. Methylxanthines have no routine role in bronchiectasis.
    • Inhaled corticosteroids: inhaled steroids should not be used routinely in children with bronchiectasis and current evidence does not support routine use of inhaled corticosteroids in adults with bronchiectasis (unless the child or adult also has asthma).
    • There is no evidence for a role for leukotriene receptor antagonists or other anti-inflammatory drugs in bronchiectasis.
    • Oxygen therapy:
      • The use of oxygen therapy may need to be considered.
      • Noninvasive ventilation can improve quality of life in some patients with chronic respiratory failure due to bronchiectasis.[1]

Surgery

  • In some cases, especially advanced or complicated disease, surgical resection of damaged lung may be beneficial.[13]
  • Lung resection surgery may be considered in patients with localised disease in whom symptoms are not controlled by medical treatment.[1]
  • Bronchial artery embolisation and/or surgery is first-line therapy for the management of massive haemoptysis.[1]
  • Lung transplantation may need to be considered if pulmonary function is very poor with FEV1 below 30% of predicted.[14]
  • Before antibiotics, death would occur within 5 years but now, with aggressive therapy, the outcome is much better. However, there continues to be a significant reduction in life expectancy in patients with bronchiectasis.[1]
  • Good nutrition to maintain an ideal body weight and regular vaccination correlate with improved survival.[15]
  • Hypoxaemia, hypercapnia, degree of dyspnoea, and radiological extent of disease are closely correlated with mortality.[15]
  • Chronic colonisation by P. aeruginosa, severe exacerbations, and systemic inflammation (raised C-reactive protein) are associated with disease progression in non-cystic fibrosis bronchiectasis.[16]

Further reading & references

  1. Guideline for non-CF Bronchiectasis, British Thoracic Society (July 2010)
  2. Lazarus A, Myers J, Fuhrer G; Bronchiectasis in adults: a review. Postgrad Med. 2008 Sep;120(3):113-21.
  3. Angrill J, Agusti C, Torres A; Bronchiectasis. Curr Opin Infect Dis. 2001 Apr;14(2):193-7.
  4. O'Donnell AE; Bronchiectasis. Chest. 2008 Oct;134(4):815-23.
  5. Morrissey BM, Harper RW; Bronchiectasis: sex and gender considerations. Clin Chest Med. 2004 Jun;25(2):361-72.
  6. Pasteur MC, Helliwell SM, Houghton SJ, et al; An investigation into causative factors in patients with bronchiectasis. Am J Respir Crit Care Med. 2000 Oct;162(4 Pt 1):1277-84.
  7. ten Hacken NH, van der Molen T; Bronchiectasis. BMJ. 2010 Jul 14;341:c2766. doi: 10.1136/bmj.c2766.
  8. Murray MP, Hill AT; Non-cystic fibrosis bronchiectasis. Clin Med. 2009 Apr;9(2):164-9.
  9. Rosen MJ; Chronic cough due to bronchiectasis: ACCP evidence-based clinical practice guidelines. Chest. 2006 Jan;129(1 Suppl):122S-131S.
  10. Smith IE, Flower CD; Review article: imaging in bronchiectasis. Br J Radiol. 1996 Jul;69(823):589-93.
  11. Chang CC, Morris PS, Chang AB; Influenza vaccine for children and adults with bronchiectasis. Cochrane Database Syst Rev. 2007 Jul 18;(3):CD006218.
  12. Chang CC, Singleton RJ, Morris PS, et al; Pneumococcal vaccines for children and adults with bronchiectasis. Cochrane Database Syst Rev. 2009 Apr 15;(2):CD006316.
  13. Agasthian T, Deschamps C, Trastek VF, et al; Surgical management of bronchiectasis. Ann Thorac Surg. 1996 Oct;62(4):976-8; discussion 979-80.
  14. Fischer S, Struber M, Haverich A; Current status of lung transplantation: patients, indications, techniques and outcome. Med Klin (Munich). 2002 Mar 15;97(3):137-43.
  15. Onen ZP, Gulbay BE, Sen E, et al; Analysis of the factors related to mortality in patients with bronchiectasis. Respir Med. 2007 Jul;101(7):1390-7. Epub 2007 Mar 19.
  16. Martinez-Garcia MA, Soler-Cataluna JJ, Perpina-Tordera M, et al; Factors associated with lung function decline in adult patients with stable Chest. 2007 Nov;132(5):1565-72.

Disclaimer: This article is for information only and should not be used for the diagnosis or treatment of medical conditions. EMIS has used all reasonable care in compiling the information but make no warranty as to its accuracy. Consult a doctor or other health care professional for diagnosis and treatment of medical conditions. For details see our conditions.

Original Author:
Dr Colin Tidy
Current Version:
Last Checked:
26/10/2010
Document ID:
1888 (v21)
© EMIS