Bronchiectasis

 

Bronchiectasis is a persistent or progressive condition characterized by dilated, thick-walled bronchi, which can affect both children and adults.  Symptoms can vary from intermittent expectoration and infection, to persistent daily expectoration of large volumes of purulent sputum.  Bronchiectasis can be limited to one area of a lung (focal), or can be widespread (diffuse).  The most common cause is previous severe lower respiratory tract infection such as pneumonia, pertussis, pulmonary tuberculosis, mycoplasma, influenza, and other viral infections, however approximately 40% of adults and children with bronchiectasis have no clear initiating event or underlying cause

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Suspected bronchiectasis

Assessment

Suspect bronchiectasis in adults with a persistent productive cough, particularly in the presence of any of the following:

  1. Daily expectoration of large volumes of purulent sputum
  2. Breathlessness
  3. Haemoptysis which can be frank (up to 10 ml) or massive (more than 235 mL)
  4. Chest pain that is present between exacerbations and is usually non-pleuritic
  5. Sputum colonization with Pseudomonas aeruginosa
  6. Young age at presentation
  7. History of symptoms over many years
  8. Absence of smoking history

On examination there might be:

  1. Coarse crackles, especially in the lower lung zones
  2. Wheeze
  3. Large airway rhonchi (low pitched snore-like sounds)
  4. Finger clubbing

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Suspect bronchiectasis in children with:

  1. A chronic moist or productive cough, especially between viral colds, or with positive sputum cultures for Staphylococcus aureusHaemophilus influenzaePsuedomonas aeruginosa, non-tuberculosis mycobacteria, or Burkholderia cepacia complex
  2. Asthma that does not respond to treatment
  3. An episode of severe pneumonia, especially if symptoms, physical signs, or radiological changes do not completely resolve
  4. Recurrent pneumonia
  5. Pertussis-like illness that fails to resolve after 6 months
  6. Persistent and unexplained physical signs or chest radiographic abnormalities
  7. Localised chronic bronchial obstruction
  8. Unexplained haemoptysis
  9. Exertional breathlessness

On examination, there might be:

  1. Persistent inspiratory crackles
  2. Wheeze
  3. Finger clubbing
  4. Chest hyperinflation
  5. Cyanosis

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Consider alternative diagnoses and be aware that bronchiectasis may co-exist with other conditions including chronic obstructive pulmonary disease (COPD) and asthma

Arrange investigations to help rule out other conditions

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Investigations and referral

  1. If bronchiectasis is suspected, arrange the following investigations:
    1. Sputum culture — to identify colonizing pathogens
    2. Chest X-ray — to exclude other pathology and to help confirm the diagnosis where disease is severe
    3. Post-bronchodilator spirometry — to assess the severity of airflow obstruction
    4. Other investigations — guided by clinical findings to confirm or rule out underlying causes for bronchiectasis
  2. Assess for the presence of anxiety or depression, particularly in people who have severe breathlessness
  3. Document the person's smoking history. If they currently smoke, offer support to stop
  4. Calculate the person's body mass index (weight in kg/height in m2). If overweight, encourage weight loss
  5. Refer all people with suspected bronchiectasis to a respiratory consultant for investigations to confirm the diagnosis and determine the underlying cause, and for appropriate treatment

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Follow up for confirmed bronchiectasis

NOTE:  If the disease is stable, with minimal exercise limitation and few exacerbations, follow-up is likely to be exclusively in primary care

NOTE:  For people with intermediate disease severity, follow-up is usually shared between primary care and secondary care

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  1. All adults with bronchiectasis should be offered an annual review in primary care. During each review, ask about:
    1. Smoking  Offer people who smoke advice and support to help them stop
    2. The number of exacerbations in the last year  Refer people with three or more infective exacerbations a year, and people with fewer exacerbations that are causing significant morbidity to a respiratory specialist. These people might be considered for long-term prophylactic antibiotic treatment
    3. Breathlessness associated with activities of daily living  Refer people with breathlessness for pulmonary rehabilitation
    4. Sputum volume and character  Send sputum for culture and sensitivity at annual review or if it has become persistently purulent between exacerbations. Specialist follow up is required for people with chronic colonization with Pseudomonas aeruginosa, opportunist Mycobacteria, or meticillin-resistant Staphylococcus aureus (MRSA)
  2. Do not routinely repeat chest X-rays
  3. Routine annual spirometry is not recommended for people who are stable, with minimal exercise limitation, and who have few exacerbations
  4. Ensure all people with bronchiectasis:
    1. Understand the condition and know how to recognize exacerbations
    2. Have been taught an airway clearance technique by a physiotherapist — Refer the person to a physiotherapist if they have not been taught an airway clearance technique
  5. Offer immunization against Streptococcus pneumoniae and seasonal influenza
  6. Consider checking vitamin D levels in people diagnosed with bronchiectasis
  7. Regular specialist follow up is required for all children with bronchiectasis, and adults with:
    1. Deteriorating bronchiectasis with declining lung function
    2. Bronchiectasis requiring long-term prophylactic antibiotics
    3. Bronchiectasis associated with rheumatoid arthritis, immune deficiency, inflammatory bowel disease, primary ciliary dyskinesia, and allergic bronchopulmonary aspergillosis
    4. Advanced disease

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Infective exacerbation of bronchiectasis

Symptoms

Symptoms of an infective exacerbation of bronchiectasis include acute deterioration of normal symptoms (usually over several days) such as:

  • Worsening cough
  • Increased sputum volume, viscosity, or purulence
  • Increased breathlessness

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Some people might also develop new symptoms such as haemoptysis, fever, or pleurisy

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Management and prescribing

Arrange hospital admission for adults who:

  • Are unable to cope at home
  • Are cyanosed or confused
  • Have a respiratory rate of more than 25 breaths per minute
  • Have signs of cardiorespiratory failure (such as marked breathlessness, rapid respiration, laboured breathing, cyanosis, or worsening peripheral oedema)
  • Have a temperature of 38°C or more
  • Are unable to take oral therapy
  • Have failed to respond adequately to oral therapy
  • Have pleuritic pain severe enough to inhibit coughing and the clearing of secretions

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Arrange hospital admission for children who:

  • Are cyanosed
  • Have increased respiratory rate and work of breathing
  • Have signs of cardiorespiratory failure (such as marked breathlessness, rapid respiration, laboured breathing, cyanosis, or worsening peripheral oedema)
  • Have a temperature of 38°C or more
  • Are unable to take oral therapy
  • Have failed to respond adequately to oral therapy

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For people not requiring hospital admission:

  1. Send sputum for culture and sensitivity testing before starting antibiotics.  If the person is taking long-term antibiotics, advise them to stop
  2. Prescribe an antibiotic for 10-14 days.  Do not await the results of culture
    1. The presence of mucopurulent or purulent sputum alone without deterioration in symptoms is not necessarily an indication for antibiotic treatment, particularly in adults
  3. Prescribe short-acting inhaled β2 agonist (such as salbutamol) if necessary for wheeze or breathlessness in the acute phase
  4. Do not prescribe inhaled or oral corticosteroids, unless required for the treatment of coexisting asthma or chronic obstructive pulmonary disease (COPD)
  5. Ensure that a suitable airway clearance technique (that has been taught by a respiratory physiotherapist) is used during the exacerbation
    1. Arrange an urgent appointment with a physiotherapist if the person has not already been taught this or if they cannot manage this alone
  6. Review the response to empirical treatment when sputum culture and sensitivity results are available
    1. If the person is responding well, continue with the prescribed antibiotic.  Do not change the treatment based on the culture results
    2. If the person has not responded well to treatment, prescribe a different antibiotic.  The choice of antibiotic should be guided by the results of sputum culture and sensitivity testing
  7. If the person deteriorates at any stage after starting treatment, re-assess to see if hospital admission is indicated

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If the person was previously taking long-term antibiotics, after the exacerbation has been treated, prescribe a prophylactic dose of a new antibiotic based on the results of sputum culture and sensitivity testing, and contact their respiratory physician to notify them of the change in antibiotics

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Antibiotic choice

Previous microbiology cultures (where available) should guide antibiotic choice

  • For adultsClick here for the table, taken from the British Thoracic Society, which lists the recommended antibiotics for acute exacerbations of bronchiectasis if results from a previous sputum sample are available
  • For children - See the British National Formulary (BNF) for dosage and use doses for severe infection

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When previous microbiology culture are not available:

  1. Prescribe to local protocols where available, or
  2. Prescribe amoxicillin 500 mg three times a day for 10-14 days
  3. Alternatives for people with a true allergy to penicillin are clarithromycin 500 mg twice a day, erythromycin 500 mg four times a day, or doxycycline (adults only) 200 mg stat and then 100 mg once a day (all for 10-14 days).  Erythromycin is the macrolide of choice in pregnant and breastfeeding women

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Follow up

  1. Provide a self-management plan that provides personalized advice on:
    1. Lifestyle, including dietexercise, and smoking cessation (if necessary)
    2. Recognizing early signs of an exacerbation such as:
      1. Increased cough and/or breathlessness
      2. Increased sputum, discoloured sputum, or change of viscosity
      3. Haemoptysis
      4. Fever
  2. Ensure that the person knows when to take any rescue medication that has been prescribed by a specialist (if applicable)
  3. Provide advice about sources of information and support for people with bronchiectasis, such as the British Lung Foundation

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Microbiology

Please send sputum cultures to the Microbiology Department at East Kent Hospitals (find details here)

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Chest radiography

For chest x-rays, you can refer the patient to the Radiology Department at East Kent Hospitals (find details here)

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Spirometry

In combination with undertaking structured clinical assessment of the patient's signs and symptoms, GPs should carry out quality-assured spirometry.  Spirometry is performed in primary care and must be carried out by suitably trained nurses accredited to ARTP standards.  Please click on the Spirometry map register for a full list of those practices where spirometry is being undertaken to the accredited standard

Further information:

Advice and Guidance is being made available for all specialties, and is being provided by consultant specialists at East Kent Hospitals.  To make a request or to check to if a query has been answered, you will need to log in via the electronic Referral System (eRS)

Click here for the "how to access" e-Referral Advice and Guidance Manual for instructions on how to make a request and check responses

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