Bronchiectasis and chronic suppurative lung disease
Author Professor Anne Chang
The customary diagnosis of bronchiectasis, usually defined as irreversible airway dilatation, is based solely on high-resolution chest computed tomography scans (HRCT). The use of a radiological definition is problematic for several reasons, particularly clinically for Aboriginal and Torres Strait Islander people living in remote areas.94,95 Thus, in the clinical guideline specific for Aboriginal and Torres Strait Islander people living in remote regions94 and the national guidelines,2,95 the preferable term is chronic suppurative lung disease (CSLD) when symptoms and/or signs of bronchiectasis are present with or without HRCT features. These symptoms and/or signs are: continuous, wet or productive cough for >8 weeks ± other features such as exertional dyspnoea, reactive airway disease, recurrent chest infections, growth failure, clubbing, hyperinflation or chest wall deformity.95 Bronchiectasis refers to CSLD with the presence of HRCT radiological features.95
The incidence of CSLD has declined over the past century. However, in the past decade, it is increasingly recognised as an important contributor to chronic respiratory morbidity in both Aboriginal and Torres Strait Islander people94 and other children and adults in Australia96,97 and globally.98 It is also increasingly recognised as an alternative or concomitant diagnosis to common respiratory conditions such as ‘difficult asthma’ and COPD.99 Two studies have described a prevalence of bronchiectasis in COPD at 29%99 and 50%.100 In a cohort of newly referred adults with ‘difficult asthma’, bronchiectasis was detected in 40%.101
In Aboriginal and Torres Strait Islander people, CSLD 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 years102 and the prevalence is 1 in every 68 children aged <15 years.103 Hospitalisation rates for CSLD is increasing in Queensland (age standardised rate of ~65 per 100 000 in 2005 to ~90 per 100 000 in 2009),104 with the rate in Aboriginal and Torres Strait Islander people about 2.7 times that for non-Indigenous Queenslanders in 2009.104 There are no data for urban dwelling Aboriginal and Torres Strait Islander people but 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.105
The morbidity of people with bronchiectasis include increased hospitalisation, excess days off work/school, poor quality of life and complications associated with chronic cough.106–108 Complications associated with bronchiectasis extend beyond the respiratory system and include cardiac problems (eg. impaired left ventricular diastolic function,199 cor pulmonale, systemic effects (eg. reduced wellbeing and increased acute phase reactants),110 sleep disturbance96 and psychological difficulties associated with anxiety and depression.111,112 Furthermore, chronic endobronchial infection, which is present in CSLD, is an independent risk factor for atherosclerosis, coronary heart disease and coronary deaths.113,114
The only published Australian mortality data for bronchiectasis are from a central Australian hospital based cohort of 61 adults (97% were Aboriginal) in which 11.5% of people had died within 12 months.115 The cohort comprised predominantly middle aged adults (mean age 42 + SD 15 years) and most had not received standard care: only 13 (21.3%) had lung function tests performed).115 Overseas, mortality rates vary widely from a 4 year survival of 58% (Turkey) to 75% survival at 8.8 years (Finland).116
Despite the considerable prevalence and disease burden, services for CSLD receive disproportionately fewer allocated resources when compared with other chronic respiratory diseases.117–119 Effective clinical management reduces both short103,120 and long term morbidity associated with CSLD.121,122 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.122–124 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 CSLD95) 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 1 second (FEV1).125 Optimal overall management and treatment can potentially prevent chronic respiratory disease in a substantial number of people.126 Although robust trials are needed, primary healthcare providers can play a crucial role in the recognition and early detection of disease as well as the long term management to prevent complications and premature death.95
Immunisation (pneumococcal, influenza) is effective in preventing severe and recurrent acute respiratory illnesses (ARIs),127 however data of its effectiveness specific to the Aboriginal and Torres Strait Islander health context are lacking.128 Delayed immunisation129 is one postulate why pneumococcal conjugate-7 vaccine has not reduced ARIs in Aboriginal and Torres Strait Islander children. This is in contrast to global data that have shown substantial ARI reduction post pneumococcal vaccination.127
Aboriginal and Torres Strait Islander children hospitalised with pneumonia were 15 times more likely to develop bronchiectasis and for recurrent pneumonia the risk increases further.130 In a cohort study, 25.6% of children with hospitalised lobar pneumonia had a new diagnosed and treatable chronic respiratory illness (18% CSLD) on follow up.131 Thus, given the link between lower ARIs and bronchiectasis,130,131 as well as the association between duration of chronic cough and lung function decline in adults,125 it is good clinical practice for all children and adults with lower ARIs to be reviewed in primary care at least 3–4 weeks post episode. 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 S. pneumoniae, Moraxella catarrhalis, H. 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 2 weeks was three.132 Further, progression of illness, defined by requirement for further antibiotics, was significantly lower in the treatment group; number needed to treat was four.132
The most common symptom of CSLD is chronic cough94 and recent unpublished data have shown that Aboriginal and Torres Strait Islander children newly referred with chronic cough have a significantly higher likelihood of bronchiectasis on further assessment. As chronic cough is considered ‘normal’, it tends to be under reported by carers of Aboriginal children.133 Anecdotally, adult Aboriginal and Torres Strait Islander people also underreport their cough. In children, triggers for referral to a specialist include >2 episodes of chronic (>4 weeks) wet cough per year responding to antibiotics, and chest X-ray abnormality persisting >6 weeks following appropriate therapy.95
Frequent exacerbations, especially when hospitalisation is required, is a risk factor for lung function decline.97,134 Thus, when exacerbations are frequent (4–6 per year non-hospitalised episodes or 2 per year hospitalised episodes), consider use of maintenance antibiotics in collaboration with a specialist.
Exposure to in-utero tobacco smoke is associated with lower birthweight, increased ARI and other respiratory morbidity.135,136 Breastfeeding is protective against development of CSLD while being born premature or small for gestation is a risk factor.130 Primary prevention strategies to reduce these factors and increase breastfeeding would be beneficial (see Chapter 2: Child health). The association between poor hygiene and the excessive burden of infections (especially respiratory and gastrointestinal) has been well demonstrated.137–139 However, data specific to Aboriginal and Torres Strait Islander people as well as evidence based interventions are sparse138 (see Chapter 9: Hearing loss).
Recommendations: Bronchiectasis and chronic suppurative lung disease
|Preventive intervention type||Who is at risk?||What should be done?||How often?||Level/strength of evidence|
||All children and adults
||Ensure timely immunisation is provided
||As per National Immunisation Program Schedule (NIPS) and state/territory schedules
||People with pneumonia and lower acute respiratory infections (ARIs), particularly hospitalised episodes
||Ensure primary care provider review after the ARI episode
If wet or productive cough* is present, consider the diagnosis of CSLD.† Recommence antibiotics and undertake investigations as per management guidelines94
|3–4 weeks post episode then 2 weekly till symptoms resolve or referred
||IA (antibiotics efficacy in treatment of wet cough in children)130–132 IIIB (screening for CSLD post lower ARI episode)
|People with recurrent lower ARIs (particularly if hospitalised)
Consider a diagnosis of CSLD. Repeat chest X-ray. Refer to specialist if:
- >2 episodes of chest X-ray proven pneumonia and/or
- chest X-ray persistently abnormal for >6 weeks
||III (screening for CSLD post lower ARI episode)94,130
|People with persistent chronic (>2 months) wet cough
||Consider a diagnosis of CSLD. Assess with a chest X-ray and see above
If wet or productive cough* is present, consider the diagnosis of chronic suppurative lung disease.† Recommence antibiotics and undertake investigations as per management guidelines94
||IA (antibiotics efficacy in treatment of wet cough in children)94,132
GPP B (for effectiveness of screening and antibiotics in adults)
||Promote and encourage breastfeeding
||At postnatal checks
||IIIB (breastfeeding protective)94,130
||Promote good hygiene practices to reduce burden of infections (see Chapter 7: Hearing loss)
|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 (see Table 11.1)
||3 monthly clinic review
6 monthly specialist review
||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 (See Chapter 9: 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) (see Chapter 1: Lifestyle, smoking)
|Mothers with or at risk of having babies with low birthweights and/or premature infants
||Promote increased access to comprehensive antenatal care (see Chapter 9: Antenatal care)
IIIC (premature and low birthweight infants developing CSLD)
||People with CSLD or known bronchiectasis
||Consider maintenance antibiotics on discussion with the person’s specialist
||As per management guidelines94
|* Cough is usually underreported;40 children do not usually produce sputum and hence the term wet cough (rather than productive cough) is used94
† Bronchiectasis refers to chronic suppurative lung disease (CSLD) with the presence of high resolution chest CT (HRCT) radiological features.95 CSLD is diagnosed when symptoms and/or signs of bronchiectasis are present with or without HRCT features.95 These symptoms and/or signs are: continuous, wet or productive cough for >8 weeks, ± other features such as exertional dyspnoea, reactive airway disease, recurrent chest infections, growth failure, clubbing, hyperinflation or chest wall deformity95
Table 11.1. Regular review of bronchiectasis
|Regular review consists of:
- assessment of severity, which includes oximetry and spirometry
- sputum culture
- management of possible complications and comorbidities, particularly for gastro-oesophageal reflux disease, reactive airway disease/asthma, COPD, otorhinolaryngeal disorders and dental disease. Less commonly patients require assessments for sleep disordered breathing, cardiac complications and referral for lung transplantation
|Source: Chang AB, Bell SC, Byrnes CA, Grimwood K, Holmes PW, King PT, et al 201095
Resources for health professionals (Lung Foundation)
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