Recommendations: Bronchiectasis and chronic suppurative lung disease
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Preventive intervention type
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Who is at risk?
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What should be done?
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How often?
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Level/ strength of evidence
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References
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Immunisation
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All children and adults, including pregnant women |
Ensure timely immunisation is provided |
As per National Immunisation Program Schedule (NIPS) and state and territory schedules |
IA |
1 |
Screening
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People with pneumonia and lower acute respiratory infections(ARIs) (particularly hospitalised episodes) |
Ensure primary healthcare providers review the patient after the ARI episode
If wet or productive cough* is present, consider the diagnosis of bronchiectasis/ chronic suppurative lung disease (CSLD).‡ Recommence antibiotics and undertake investigations as per management guidelines (refer to ‘Resources’) or refer to a specialist (Box 2)1 |
3–4 weeks postepisode, then two-weekly until symptoms resolve or the patient is referred |
IA (antibiotics efficacy in treatment of wet cough in children)
III–IIB (screening for bronchiectasis post– lower ARI episode) |
37, 38, 39 |
People with recurrent lower ARIs (in children, this is >2 episodes of hospitalised chest X-ray proven pneumonia ever), and/or with persistent chronic (>4 weeks) wet cough† |
Consider a diagnosis of bronchiectasis. Repeat a chest X-ray
Refer children to a specialist if there is persistent wet cough and/or abnormal CXR (Box 2) |
Opportunistic |
III–II (screening for bronchiectasis post– lower ARI episode)
IA (antibiotics efficacy in treatment of wet cough in children)
GPP B (for effectiveness of screening and antibiotics in adults) |
1, 37, 39, 40, 47 |
People with history of tuberculosis |
Clinically assess for chronic lung disease symptoms,‡ and undertake spirometry |
Opportunistic |
III–II |
48 |
Adults with chronic obstructive pulmonary disease (COPD) |
Undertake spirometry (refer to Chapter 9: Respiratory health, ‘Chronic obstructive pulmonary disease’). Assess for bronchiectasis symptoms and consider referral to specialist if:
- there is a history of daily sputum production
- sputum has persistent infection, especially with Pseudomonas aureginosa
- there are increasing exacerbations
- there is lung function decline
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Opportunistic |
III–II (screening for bronchiectasis in adults with COPD) |
7, 8, 49 |
Behavioural
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All Infants |
Promote and encourage breastfeeding |
At postnatal checks |
III–IIB (breastfeeding protective) |
1, 37 |
All children |
Promote good hygiene practices to reduce burden of infections (refer to Chapter 7: Hearing loss) |
Opportunistic |
GPP B |
45 |
People with CSLD or known bronchiectasis |
Assess cough severity, quality of life, and exacerbating factors.
Undertake regular review to prevent and manage complications and comorbidities (Box 1) |
Three-monthly clinic review
Six-monthly specialist review |
GPP B |
1, 2 |
Infants at risk of exposure to environmental tobacco smoke both in-utero and in the postnatal period |
Advise and assist pregnant women to avoid smoking (refer to Chapter 2: Antenatal care)
Advise parents/carers who smoke about the harms of environmental tobacco smoke and the need to limit childhood exposure, particularly in confined spaces (eg homes and motor vehicles) (refer to Chapter 1: Lifestyle, ‘Smoking’) |
Opportunistic |
IIIC |
43, 44 |
Mothers with, or at risk of having, babies with low birth weights and/ or premature infants |
Promote increased access to comprehensive antenatal care (refer to Chapter 2: Antenatal care) |
Opportunistic |
GPP
III–IIC (premature and low birth weight infants developing CSLD) |
1, 6, 37 |
People with CSLD or known bronchiectasis |
Consider maintenance antibiotics on discussion with the person’s specialist |
As per clinical practice guidelines1 |
IA |
50, 51 |
Chemo-prophylaxis
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*Cough is usually underreported.41
†Children do not usually produce sputum and hence the term ‘wet cough’ (rather than ‘productive cough’) is used.1
‡Bronchiectasis refers to symptoms of CSLD in the presence of high-resolution computed tomography (HRCT) chest scan findings of airway dilatation when clinically stable.2 CSLD is diagnosed when symptoms and/or signs of bronchiectasis are present without availability of an HRCT to confirm bronchiectasis, or, in children, without the HRCT features of bronchiectasis.2 These symptoms and/or signs are recurrent (>3 episodes) wet or productive cough, each lasting for >4 weeks, with or without other features (eg exertional dyspnoea, symptoms of airway hyper-responsiveness, recurrent chest infections, growth failure, digital clubbing, hyperinflation or chest wall deformity).2 |
Box 1. Reviewing patients who have chronic suppurative lung disease/bronchiectasis1
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Regular review consists of at least an annual review in adults and six-monthly in children. A multidisciplinary team is preferable, especially at the initial evaluation.
The review includes assessment of:
- severity, which includes oximetry and spirometry
- sputum culture (when available) for routine bacterial and annual mycobacterial culture
- management of possible complications and comorbidities, particularly for gastroesophageal reflux disease/aspiration, reactive airway disease/asthma, chronic obstructive pulmonary disease (COPD), otorhinolaryngeal disorders, urinary incontinence, mental health and dental disease; less commonly, patients require assessments for sleep-disordered breathing and cardiac complications
- adherence to therapies and knowledge of disease processes and treatments.
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Box 2. In children, triggers for referral to a specialist1
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Triggers include one or more of the following:
- persistent wet cough not responding to four weeks of antibiotics
- >3 episodes of chronic (>4 weeks) wet cough per year responding to antibiotics
- a chest radiograph abnormality persisting >6 weeks after appropriate therapy.
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Background
The diagnosis of bronchiectasis, defined as irreversible airway dilatation, is based largely on the findings of highresolution computed tomography (HRCT) chest scans. The use of such a radiological definition can be problematic for several reasons. This is particularly the case for Aboriginal and Torres Strait Islander people living in remote areas where access to CT scanning can be limited, and for very young children who need a general anaesthetic.1 Nonetheless, the increasing availability of CT scanners means a CT-based diagnosis should be pursued where feasible. Given these limitations, in clinical guidelines specific to Aboriginal and Torres Strait Islander people living in remote regions and in the national guidelines,1,2 an additional term of chronic suppurative lung disease (CSLD) is used when symptoms and/or signs of bronchiectasis are present without available confirmation of HRCT features.2 In this document, bronchiectasis will be used to refer to both bronchiectasis and CSLD.
The symptoms and/or signs of bronchiectasis include recurrent (>3 episodes) wet or productive cough, each lasting for >4 weeks, with or without other features including exertional dyspnoea, symptoms of airway hyper-responsiveness, recurrent lower respiratory tract infections (including pneumonia), growth failure, digital clubbing, hyperinflation and chest wall deformity.2
Bronchiectasis has had a declining incidence over the last century. However, in the last decade, it is increasingly recognised as an important contributor to chronic respiratory morbidity in both Aboriginal and Torres Strait Islander people1 and non-Indigenous children and adults in Australia,3,4 as well as globally.5,6 It is also increasingly recognised as an alternative or concomitant diagnosis to common respiratory conditions such as ‘difficult asthma’ and chronic obstructive pulmonary disease (COPD).7,8 In a cohort of newly referred adults with ‘difficult asthma’, bronchiectasis was detected in 40%.9 In part, this greater prevalence of disease may be related to the increasing availability of a highly sensitive diagnostic tool, namely HRCT. Systematic reviews have described a prevalence of bronchiectasis in COPD of 20–69%.8 However, there are no such studies among Aboriginal and Torres Strait Islander people with COPD.
In Aboriginal and Torres Strait Islander people, CSLD and bronchiectasis is anecdotally common but there is little published data. In the Northern Territory, the incidence in Aboriginal and Torres Strait Islander infants (first year of life) is 1.18 per 1000 child years (95% confidence interval [CI], 0.60–2.16)10 and the prevalence is one in every 68 children aged <15 years.11 Hospitalisation rates for patients with bronchiectasis are increasing in Queensland (age standardised rate of ~65/100,000 in 2005 to ~90/100,000 in 2009),12 with the rate in Aboriginal and Torres Strait Islander peoples about 2.7 times that for non-Indigenous Queenslanders in 2009.12 There are no data for urban-dwelling Aboriginal and Torres Strait Islander peoples, but a national multicentre study that included Central Australia, Darwin and the Torres Straits found that among children newly presenting to a respiratory service with chronic cough, Aboriginal and Torres Strait Islander children have a significantly higher incidence of radiological bronchiectasis compared to non-Indigenous children:29.4% versus 6.7% respectively, P = 0.001.13
The morbidity of people with bronchiectasis includes increased hospitalisation, excess days off work/ school, poor quality of life, and complications associated with chronic cough.14 Complications associated with bronchiectasis extend beyond the respiratory system and include cardiac problems (eg impaired left ventricular diastolic function,15 cor pulmonale), systemic effects (eg reduced wellbeing and increased acute phase reactants),16 sleep disturbance4 and psychological difficulties associated with anxiety and depression.17,18 Furthermore, chronic endobronchial infection, which is present in CSLD and bronchiectasis, is an independent risk factor for atherosclerosis, coronary heart disease and coronary deaths.19–21
The only published Australian mortality data for bronchiectasis are from Central Australian hospital-based cohorts (note: some people were in both cohorts). In the first study of 61 adults (97% were Aboriginal), 11.5% of people died within 12 months.22 The cohort comprised predominantly middle-aged adults (mean age 42 years, standard deviation [SD] 15 years), and most had not received standard care (eg only 13 [21.3%] had lung function tests performed).22 The second study23 reported the prevalence of hospitalised bronchiectasis as 103 per 10,000 population, and 34% of the cohort died at a median age of 42.5 years. Overseas, mortality rates vary widely from four year survival of 58% (Turkey) to 75% survival at 8.8 years (Finland).24
Interventions
Despite the considerable prevalence and disease burden, services for bronchiectasis are under-resourced when compared with other chronic respiratory diseases.25 The European Respiratory Society regards bronchiectasis as one of the most neglected chronic respiratory diseases.26 Effective clinical management reduces both short-term27 and long-term morbidity associated with bronchiectasis.28,29 There is increasing evidence that intensive treatment of children who either have bronchiectasis, or who are at risk of developing severe bronchiectasis, prevents poor lung function in adulthood.14,30 Cohort data have shown that approximately 80% of newly diagnosed adults with bronchiectasis were symptomatic since childhood, and that the duration of chronic cough (the most common symptom of bronchiectasis2) is inversely related to lung function at diagnosis. This means that the longer the duration of cough, the poorer the lung function at diagnosis based on forced expiratory volume at one second (FEV1).31 Optimal overall management and treatment can potentially prevent chronic respiratory disease in a substantial number of people.32 Although robust trials are needed, primary care health providers can play a crucial role in the recognition and early detection of disease as well as in long-term management to prevent complications and premature death.1
Immunisation (pneumococcal, influenza) is effective in preventing severe and recurrent acute respiratory illnesses (ARIs);33 however, data of its effectiveness specific to the Aboriginal and Torres Strait Islander health context are lacking.34 Delayed immunisation (ie poor timeliness)35 is one postulate why 7-valent pneumococcal conjugate vaccine (7vPCV) did not reduce ARIs in Aboriginal peoples and Torres Strait Islander children. This is in contrast to global data that has shown substantial ARI reduction post–PCV.36
Aboriginal and Torres Strait Islander children hospitalised with pneumonia are 15 times more likely to develop bronchiectasis than non-Indigenouschildren, and for recurrent pneumonia the risk increases further.37 One cohort study described that 25.6% of Aboriginal children hospitalised for lobar pneumonia had a new diagnosis and treatable chronic respiratory illness (18% bronchiectasis) on follow-up.38 In a follow-up study of Aboriginal children hospitalised with bronchiolitis, the presence of cough at three weeks postdischarge increased the odds of having bronchiectasis diagnosed by 13 months.39 Differentiating between bronchiectasis as a cause or consequence of an earlier admission diagnosed as pneumonia or bronchiolitis can be problematic. Nevertheless, given the likely link between lower ARIs and bronchiectasis,37,38 as well as the association between duration of chronic cough and lung function decline in adults,31 it is good clinical practice for all children and adults with lower ARIs to be reviewed in primary care at least three to four weeks post-ARI, especially post-hospitalisation. They should be screened for the presence of chronic cough and persistence of other respiratory symptoms and signs (eg wheeze and crackles in chest auscultation).
When chronic (>4 weeks) wet cough is present, appropriate antibiotics (covering common respiratory pathogens – Streptococcus pneumoniae, Moraxella catarrhalis, Haemophilus influenzae) are recommended. In a Cochrane meta-analysis, the cure rate in children who received antibiotics was significantly higher than in controls, and the number of children needed to treat to demonstrate a benefit at two weeks was three.40 Further, progression of illness, defined by requirement for further antibiotics, was significantly lower in the treatment group.40
The most common symptom of CSLD/bronchiectasis is chronic cough.1 Data has shown that Aboriginal people and Torres Strait Islander children newly referred with chronic cough have a significantly higher likelihood of bronchiectasis on further assessment.13 As chronic cough is considered ‘normal’, it tends to be underreported by carers of Aboriginal children.41 Anecdotally, adult Aboriginal people and Torres Strait Islanders also under-report their cough. In children, triggers for referral to a specialist include:
- >3 episodes of chronic (>4 weeks) wet cough per year responding to antibiotics
- chest X-ray abnormality persisting >6 weeks following appropriate therapy (Box 2).2 Frequent exacerbations of bronchiectasis, especially when hospitalisation is required, is a risk factor for lung function decline.3,42 Thus when exacerbations are frequent (four or more per year non-hospitalised episodes, or two per year hospitalised episodes), consider use of maintenance antibiotics in collaboration with specialists.1
Exposure to in-utero tobacco smoke is associated with lower birth weights, increased ARI and other respiratory morbidity.43,44 Breastfeeding is protective against development of bronchiectasis, while being born premature or small for gestation is a risk factor.37 Primary prevention strategies to reduce these factors and increase breastfeeding would be beneficial (refer to Chapter 3: Child health). The association between poor hygiene and the excessive burden of infections (especially respiratory and gastrointestinal) has been well demonstrated.45,46 However, data specific to Aboriginal and Torres Strait Islander peoples as well as evidence-based interventions are sparse45 (refer also to Chapter 7: Hearing loss).
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