g logo ipb green

Guideline for non-CF bronchiectasis

What are the pathology and underlying causes?

  • Bronchiectasis is a persistent or progressive condition characterised by dilated thick-walled bronchi. The symptoms vary from intermittent episodes of expectoration and infection localised to the region of the lung that is affected to persistent daily expectoration often of large volumes of purulent sputum
  • Bronchiectasis may be associated with other non-specific respiratory symptoms including dyspnoea, chest pain, and haemoptysis, and may progress to respiratory failure and cor pulmonale
  • The underlying pathological process is damage to the airways which results from an event or series of events where inflammation is central to the process
  • Congenital defects should be considered in all patients with bronchiectasis
  • Gastric aspiration should be considered as a cause in all patients
  • A history of previous severe lower respiratory tract infections due to bacterial and viral pneumonia, pertussis, or tuberculosis should be sought in all patients with bronchiectasis
  • Where possible, the temporal relationship of identified infections to the onset of chronic respiratory symptoms should be determined
  • Identifying a post-infectious cause may limit the need for further investigations, particularly in elderly subjects

Immune deficiency and bronchiectasis

  • The possibility of underlying immune deficiency, particularly antibody deficiency, should be considered in all children and adults with bronchiectasis
  • Serious, persistent, or recurrent infections, particularly involving multiple sites or infections with opportunist organisms should raise the suspicion of immune deficiency
  • The possibility of symptomatic or clinically silent bronchiectasis should be considered as a potential complication in all patients with immune deficiency, particularly primary antibody deficiency
  • In patients with immune deficiency and patients with bronchiectasis, features in the history or clinical examination which may support the co-existence of both conditions should be considered and adequately assessed
  • In patients with suspected or proven immune deficiency and bronchiectasis in combination, specialist aspects of diagnosis, monitoring, and management should optimally be provided within a shared specialist care arrangement

What is the relationship of other airway diseases to bronchiectasis?

  • All patients with bronchiectasis should be assessed for evidence of allergic bronchopulmonary aspergillosis (ABPA), which is a clinical diagnosis based on presentation and immunological tests (Aspergillus -specific IgE and IgG)
  • In adults, asthma should be considered as the cause of bronchiectasis if no other cause is identified
  • The possibility of diffuse panbronchiolitis should be considered in patients of Far-Eastern ethnic origin
  • For all patients with bronchiectasis, the possibility of underlying cystic fibrosis should be considered

Which connective tissue disorders are associated with bronchiectasis?

  • A history of rheumatoid arthritis should be sought in all patients with bronchiectasis
  • Closer follow-up of patients with rheumatoid arthritis-related bronchiectasis is warranted in view of a poorer prognosis

Inflammatory bowel diseases

  • Bronchiectasis should be considered in patients with inflammatory bowel disease who develop a chronic productive cough

Disorders of ciliary function

  • In all children with bronchiectasis, a detailed history of the neonatal period should be taken
  • In children and adults with bronchiectasis, a history of chronic upper respiratory tract problems, particularly otitis media, should be sought
  • Adults should be questioned about any history of infertility
  • Routine screening for α1 -antitrypsin deficiency is not required unless the radiological investigations suggest basal emphysema
  • The assessment of patients with bronchiectasis should include a search for features of yellow nail syndrome
  • Every patient with bronchiectasis should have an assessment of upper respiratory tract symptoms

Clinical assessment and investigations

Which children should be investigated for bronchiectasis?

  • Consideration should be given to evaluating a child for bronchiectasis who presents with:
    • chronic moist/productive cough, especially between viral colds or with positive bacterial cultures
    • asthma that does not respond to treatment
    • a single positive sputum culture, in the setting of chronic respiratory symptoms, for Staphylococcus aureus, Haemophilus influenzae, Pseudomonas aeruginosa, non-tuberculous mycobacteria, or Burkholderia cepacia complex
    • an episode of severe pneumonia, particularly 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 chest radiographic abnormalities
    • 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 (PCD), or immunodeficiency

Which adults should be investigated for bronchiectasis?

  • Bronchiectasis should be considered in all adults who have:
    • persistent productive cough. Factors favouring further investigation are 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 non-productive cough
    • patients thought to have chronic obstructive pulmonary disease may have bronchiectasis alone or in addition and referral for investigation is appropriate if:
      • management is not straightforward
      • there is slow recovery from lower respiratory tract infections
      • recurrent exacerbations
      • there is no history of smoking

What are the symptoms and signs of bronchiectasis in children?

  • Respiratory symptoms, particularly cough and sputum production, should be assessed and recorded in all children with bronchiectasis
  • There should be a high index of suspicion for diagnosing bronchiectasis in children with chronic respiratory symptoms
  • The finding of persistent lung crackles on auscultation should alert the clinician to possible underlying bronchiectasis

What symptoms and signs should be assessed in an adult with bronchiectasis?

  • 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

Investigations directed at underlying cause

  • Investigations should be performed to establish the cause and severity of disease

What blood tests should be performed?

  • The following tests should be performed in all patients:
    • full blood count and white cell differential erythrocyte sedimentation rate or C-reactive protein; routine biochemistry
  • The following should be measured in all patients:
    • serum immunoglobulins (IgG, IgA, IgM) and serum electrophoresis
    • serum IgE, Aspergillus fumigatus radioallergosorbent test (RAST)/catabolite activator protein (CAP), and Aspergillus precipitins

What immunological tests should be done on all patients?

  • 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
  • Where screening tests or clinical presentation indicate that further immunological investigation is warranted, this should be planned and undertaken within an agreed and integrated respiratory/immunology protocol

What are the second-line immunological investigations and when should they be performed?

  • Consideration of second-line assessment of immune competence is necessary in the following circumstances:
    • antibody screening investigations have demonstrated the presence of an antibody deficiency disorder (to refine diagnosis, detect immune complications, and plan treatment)
    • 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 telangiectasis, eczema, dermatitis, petechiae, manifestations of endocrinopathy, unexplained failure to thrive, enlargement of 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 (e.g. lobectomy, tonsillectomy, insertion of grommets, incision of boils), are persistent or recurrent despite multiple or prolonged courses of antibiotics, involve unusual/opportunist micro-organisms, or involve multiple sites (e.g. sinuses or middle ear in addition to the bronchial tree)

Sputum microbiology

  • All children and adults with bronchiectasis should have an assessment of lower respiratory tract microbiology
  • Persistent isolation of S aureus (and/or P aeruginosa in children) should lead to consideration of underlying ABPA or cystic fibrosis
  • Respiratory tract specimens should be obtained in all patients with bronchiectasis
  • To maximise the chances of isolating H influenzae and Streptococcus pneumoniae, specimens should reach the microbiology laboratory within 3 hours

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 (FEF25–75) should be measured at initial assessment
  • All adults with bronchiectasis should have measures of FEV1, FVC, and peak expiratory flow (PEF)
  • Repeat assessment ofFEV1, FVC, and PEF should be made at least annually in those patients attending secondary care
  • Patients with immune deficiency or PCD should have measurements ofFEV1 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 chronic obstructive pulmonary disease/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

Management: principles and general approach

General approach and treatment of the specific underlying cause

  • Identify and treat underlying cause to prevent disease progression
  • Maintain or improve pulmonary function
  • Reduce exacerbations
  • Improve quality of life by reducing daily symptoms and exacerbations
  • In children, achieve normal growth and development
  • Patients with primary or secondary immune deficiency should be under joint care with a clinical immunologist
  • Patients with cystic fibrosis should be referred to a cystic fibrosis specialist centre

Role of primary care

What is the interface between primary and secondary care?

  • Patients who should have regular follow-up in secondary care include:
    • all children with bronchiectasis
    • patients with chronic P aeruginosa, opportunist mycobacteria, or methicillin-resistant S aureus colonisation
    • deteriorating bronchiectasis with declining lung function
    • recurrent exacerbations (>3 per year)
    • patients receiving prophylactic antibiotic therapy (oral or nebulised)
    • patients with bronchiectasis and associated rheumatoid arthritis, immune deficiency, inflammatory bowel disease, and PCD
    • patients with ABPA
    • patients with advanced disease and those considering transplantation
    • patients with bronchiectasis should as a minimum be referred to a chest physician, physiotherapist, and respiratory nurse with expertise in the condition

Role of nurses

What role do nurses play in the management of bronchiectasis?

  • Primary and secondary care nurses should receive training in the management of bronchiectasis

Education

  • Give a written explanation of bronchiectasis and the role of infection in exacerbations
  • Record where there is an identified cause and explain what this is and how it will be treated
  • Explain treatment approaches including airway clearance techniques, airway therapies, and management of infections
  • Explain how to recognise an exacerbation
  • Give information on how to access medical care in the event of an exacerbation (it may be appropriate for antibiotics to be kept in reserve at home and for telephone contact to be sufficient)
  • Explain the usefulness of sending a sputum sample for culture and sensitivity to aid appropriate management with antibiotics
  • Give information on how to access British Thoracic Society (BTS) guidelines
  • An individual plan for follow-up and monitoring detailing patient/parent role in monitoring symptoms, GP role in monitoring, and hospital specialist role may be useful
  • Children with PCD should be referred to a specialist centre
  • Give advice regarding pneumococcal vaccination and annual flu vaccination

Disease monitoring

  • The following information should be recorded whether the patient is seen in primary or secondary care:
    • spirometry (at least annually)
    • number of exacerbations and which antibiotics were taken in follow-up period
    • estimated sputum volume per day and sputum character
    • result of sputum culture
    • usual daily symptoms of cough, sputum, and general wellbeing (tiredness, malaise), and the degree of disturbance of activities of daily life
    • concordance with treatment prescribed
    • specific concerns from the patient or parent

Airway pharmacotherapy

Are bronchodilators of use in bronchiectasis?

  • It seems appropriate to assess patients with airflow obstruction for reversibility to ß2 agonist and anticholinergic bronchodilators and to institute therapy where lung function or symptoms improve on therapy

Are inhaled corticosteroids a useful treatment for bronchiectasis?

  • Inhaled steroids should not be used routinely in children with bronchiectasis (outside of use for those patients with additional asthma)
  • In adults, current evidence does not support routine use of inhaled corticosteroids in bronchiectasis (outside of use for those patients with additional asthma)

Leukotriene receptor antagonists and other anti-inflammatory agents

  • There is no evidence for a role for leukotriene receptor antagonists or other anti-inflammatory drugs in bronchiectasis

Management: antibiotic therapy

  • Previous sputum bacteriology results can be useful in deciding which antibiotic to use
  • Where possible, sputum (spontaneous or induced) or a cough swab should be obtained for culture prior to commencing antibiotics
  • Empirical antibiotics can then be started while awaiting sputum microbiology
  • In general, antibiotic courses for 14 days are standard. If there is no previous bacteriology, the first-line treatment is amoxicillin for 14 days or clarithromycin for 14 days in patients who are allergic to penicillin
  • Children not responding to empirical antibiotic courses should have an organism identified by cough swab or later by induced sputum/bronchoalveolar lavage
  • 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)

Defining and managing exacerbations (see box 1)

  • The presence of mucopurulent or purulent sputum alone or the isolation of a pathogen alone is not necessarily an indication for antibiotic treatment, particularly in adults
  • Antibiotics should be given for exacerbations that present with an acute deterioration with worsening symptoms (cough, increased sputum volume or change of viscosity, increased sputum purulence with or without increasing wheeze, breathlessness, haemoptysis) and/or systemic upset
  • 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 (e.g. 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 with cautious use in the elderly
  • Failure to respond to an antibiotic course should prompt a repeat sputum culture
  • There is no evidence to support the routine use of antiviral drugs in exacerbations

Do long-term oral antibiotics influence long-term outcome in adults?

  • Patients having ≥3 exacerbations per year requiring antibiotic therapy or patients with fewer exacerbations that are causing significant morbidity should be considered for long-term antibiotics
  • 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 should not be used until further studies are available
  • Macrolides may have disease-modifying activity and preliminary data suggest the need for a large randomised controlled trial

Do long-term nebulised antibiotics influence long-term outcome in adults?

  • Patients having≥3 exacerbations per year requiring antibiotic therapy or patients with fewer exacerbations that are causing significant morbidity should be considered for long-term nebulised antibiotics
  • In such patients, long-term nebulised antibiotics should be considered if chronically colonised with P aeruginosa. The choice of antibiotic should be guided by the antibiotic sensitivity results. Further studies are needed to address the optimal antibiotic choice and doses required

Box 1. Assessment of patients with exacerbations of bronchiectasis

Adults
Outpatients
  • History
  • Clinical examination
  • Sputum for culture (preferably prior to commencement of antibiotics)
  • Review of previous sputum microbiology
Children
Outpatients
  • Sputum for culture if spontaneous sputum (preferably prior to commencement of antibiotics)
  • If spontaneous sputum is not available, throat/cough swabs, especially if regularly repeated, may provide inferred evidence of lower respiratory tract organisms. Caution should be taken in interpreting these cultures because many of the pathogens may also be normal upper airway commensals
  • Induced sputum using hypertonic saline (3–5%) may be used to obtain lower respiratory tract samples for analysis

Definition of an exacerbation needing antibiotic therapy

Definition of an exacerbation needing antibiotic therapy

Definition of successful treatment of an an exacerbation

Definition of successful treatment of an an exacerbation

 

 

View related respiratory content

full guideline available from…
www.brit-thoracic.org.uk/document-library/clinical-information/bronchiectasis/bts-guideline-for-non-cf-bronchiectasis/

British Thoracic Society. Guideline for non-CF bronchiectasis. Thorax 2010; 65:i1–i58. 
First included: March 2016.