Chronic suppurative lung disease and bronchiectasis in children, adolescents and adults in Australia and New Zealand: TSANZ position statement summary.

Bronchiectasis is characterised by chronic cough and sputum production, recurrent respiratory infections, and computed tomography (CT) evidence of bronchial dilatation.1 Globally, bronchiectasis is the third most common chronic respiratory disorder after asthma and chronic obstructive pulmonary disease.2 In Australia, bronchiectasis is most prevalent in Indigenous populations from remote northern communities,3 where an estimated 1470 per 100 000 children aged less than 14 years1, 3 and up to 23% of Indigenous adults undergoing chest CT scans have radiographic evidence of bronchiectasis.4 Indigenous Australians with bronchiectasis die about 20 years earlier than non-Indigenous adults with bronchiectasis, who, in turn, die ten years earlier than other Australians.5 Nevertheless, bronchiectasis remains under-recognised and undertreated. An Australian cohort study reported that over 60% of referred adults with bronchiectasis had symptoms dating from childhood and a worse prognosis than those with adult-onset disease.6 In contrast, bronchiectasis in children is potentially reversible if diagnosed early and treated optimally.7, 8 Guidelines, therefore, aim for early diagnosis and treatment to reverse mild bronchiectasis in children, thereby reducing future adult bronchiectasis while seeking to preserve lung function and quality of life in others with established disease.9, 10 The 2015 Thoracic Society of Australia and New Zealand (TSANZ) guidelines11 were replaced recently by an updated TSANZ Position Statement.12 As before, the 2023 TSANZ Statement12 (Box) seeks to increase awareness of bronchiectasis in health professionals and employs the best available evidence to support its recommendations on diagnosis, underlying causes, airway clearance, controlling infection and preventing complications. Here, we discuss within the Australian context the main differences between these two publications,11, 12 followed by implementation and future research directions. Minimum baseline investigations: The TSANZ 2023 Position Statement12 was formulated by an interdisciplinary panel of health professionals caring for patients with bronchiectasis. The 2023 version12 is more inclusive than the 2015 Guideline,11 with panel members comprising nine medical practitioners, three physiotherapists, a respiratory nurse, an Indigenous academic and a health consumer representative. Several panel members had additional expertise that included infectious diseases and Indigenous and remote and rural health. For the 2023 update,12 each of the 33 statements was allocated to a panel pair (each with paediatric and adult expertise). Systematic reviews for each statement were undertaken and an updated search was performed using dates from the most recent international guidelines from the British Thoracic Society9 and the European Respiratory Society10 as a baseline. The statements were then reviewed and discussed by the whole panel before circulation as part of a Delphi process to 47 health professionals with expertise in bronchiectasis (19 were surveyed in the 2015 guideline),11 of whom 31 responded, and a final series of statements was generated. Overall, of the 33 individual statements, one is new, 28 are modified, and four unchanged from the 2015 Guideline.11 The new and modified statements are often fuller and more detailed than presented in the 2015 Guideline,11 and are accompanied by three boxes, a table and a figure.12 There is a box outlining the clinical features of bronchiectasis, including trigger points for referring children and adolescents to paediatric respiratory specialist services. Another box describes the minimum panel of investigations to perform for determining underlying aetiology, disease severity, comorbid conditions, and treatable traits. This box also describes circumstances when more extended investigations are warranted and the types of additional tests are listed. The last box presents the main objectives of optimising management for patients with bronchiectasis. The Statement's table provides an antibiotic selection guide for the empirical and directed therapy of acute respiratory exacerbations. Meanwhile, the figure is adapted from the European Respiratory Society guidelines10 and suggests a series of pathways for attempting to eradicate newly detected Pseudomonas aeruginosa in respiratory secretions. The 2023 Position Statement has an overarching technical report available in appendix 1 of the online supplement.12 The possibility of mild bronchiectasis being reversible in children if identified early and treated optimally reinforces the importance of recognising the clinical features of bronchiectasis and using paediatric radiographic diagnostic criteria when interpreting chest CT scans. However, making the diagnosis is the beginning, and not the end, of the diagnostic journey. As bronchiectasis is a heterogenous disorder, a search for an underlying cause and comorbid conditions should be initiated. This includes recognising treatable traits — that is, therapeutic targets identified by phenotypes (observable clinical characteristics) or endotypes (underlying pathobiological pathways associated with outcomes and validated biomarkers) — while determining the extent and severity of the bronchiectasis remains important. The 2023 Statement12 does this in greater detail than the 2015 Guidelines.11 It lists the possible causes of bronchiectasis and includes clinical features that should trigger additional investigations. Furthermore, as paediatric and adult patients with bronchiectasis can have different aetiologies and comorbid conditions, the baseline and extended investigations are split between these two age groups. In bronchiectasis, P. aeruginosa is associated with worse clinical outcomes,1, 7 and, consistent with other international guidelines,9, 10, 13 the 2023 Statement12 now states that eradication therapy for newly detected P. aeruginosa in respiratory secretions should be offered, rather than considered, and provides advice in the figure on how this might be achieved. This was based upon four before and after (two prospective) studies in adults reporting improved quality of life and reduced exacerbations and hospitalisations following eradication, and two systematic reviews of randomised controlled trials of inhaled antibiotics in adults with bronchiectasis and chronic P. aeruginosa infection, where eradication was associated with reduced exacerbations but was a secondary outcome.12 Although the optimal duration of antibiotic treatment of exacerbations is unknown, a single, double-blind randomised controlled trial in children and adolescents with non-severe (non-hospitalised) exacerbations found that oral amoxycillin–clavulanate was superior to placebo at achieving symptom resolution and shortening symptom duration after a 14-day treatment.14 Accordingly, the 2023 Statement12 has recommended antibiotics be continued for at least 14 days, rather than the ten days stated in the 2015 Guidelines.11 The goal of long term antibiotics (> 2 months) is to reduce bacterial load and/or airway inflammation when eradication is not possible. The recommendation for using long term oral macrolides has been simplified (considered in those with the frequent exacerbator phenotype [ie, ≥ 3 exacerbations per year], which predicts mortality, hospitalisation, and reduced quality of life)15, 16 and should be prescribed for at least a six-month trial with regular review for clinical benefit. In addition, more advice is provided on risk factors and monitoring for rare but severe adverse effects from oral macrolides. However, the advice that inhaled antibiotics should not be prescribed routinely remains unchanged, although they can be considered in adults chronically infected with P. aeruginosa and frequent exacerbations when oral macrolides are either contraindicated, not tolerated or fail to reduce exacerbation frequency. In other statements in the 2023 publication,12 electronic cigarettes are now included in products to be avoided, the indications for surgery, pre-operative assessment and where this should be performed are presented in greater detail, as are statements concerning equity of access to specialist health services, which could mean telehealth for rural and remote regions. Finally, the last and newest statement recommends planned, clearly documented and developmentally appropriate transition from paediatric to adult services, involving interdisciplinary members of both health teams. It seeks to avoid the lack of engagement and adverse health outcomes when transition arrangements are absent.17 Although endorsed statements and guidelines are produced, these are not always followed because of insufficient evidence to support their recommendations, conflicting data or a lack of awareness from inadequate dissemination.18 To help address the latter, a series of webinars is planned, and TSANZ/Australian Lung Foundation health professional and patient checklists informed by the 2023 Statement12 have been developed for distribution. A clinician- and patient-focused consensus quality standards document for managing bronchiectasis derived from the 2023 Position Statement recommendations12 is planned, using similar methodology employed by the European Respiratory Society Children's Bronchiectasis Education, Advocacy and Research Network (www.improveBE.org).19 As an initial step, the quality standards aim to further improve the health care of patients with bronchiectasis by being an advocacy tool for patients and clinicians and for monitoring health service performance.20 Despite the growing evidence base for bronchiectasis, large knowledge gaps remain and no licensed therapies exist. Consequently, guidelines often rely upon expert opinion and need regular updating. For example, a report has just been published of long term azithromycin use being most effective at reducing exacerbations between 17 and 62 weeks after its initiation in children with bronchiectasis,21 while a small retrospective case series in children with primary ciliary dyskinesia (19 had bronchiectasis) found that P. aeruginosa eradication therapy was successful in 30 of 31 infected children and sustained for at least one year in 19, but follow-up was insufficient to determine clinical benefit.22 The highly heterogenous nature of bronchiectasis is a major clinical and research challenge. This requires grouping patients by their shared pheno-endotypes using clinical, radiographic, spirometric and multiomics-derived criteria and validated biomarkers to allow treatable traits (pulmonary, aetiology, extrapulmonary, behaviour and lifestyle) to be identified and managed, as well as improving clinimetric properties in registry-based studies,16 and having well balanced arms in high quality, adequately powered randomised controlled trials.23 Additional strategies addressing heterogeneity require standardising definitions (eg, exacerbations)24 and adopting patient-focused core outcome sets (an agreed minimum set of critically important outcomes) to improve trial comparisons, meta-analyses and guidelines.25 Finally, better engagement and investment are also required in allocating resources to culturally and linguistically diverse groups, where disease burden is highest and outcomes poorest.3, 5 Future statements should depend on studies that ensured the right patient populations received the right interventions and measured the right endpoints.25 The position statement on chronic suppurative lung disease and bronchiectasis in children, adolescents and adults in Australia and New Zealand has been officially recognised as an Accepted Clinical Resource by the Royal Australian College of General Practitioners. Keith Grimwood, Maree Toombs and Anne Chang received an Australian National Health Medical Research Council (NHMRC) Centre for Research Excellence grant (1170958) for preventing and managing bronchiectasis, especially in Aboriginal and Torres Strait Islander children. Anne Chang is supported by an NHMRC Senior Practitioner Fellowship (1154302). The funding sources had no role in the planning, writing or publication of the work. Open access publishing facilitated by Griffith University, as part of the Wiley - Griffith University agreement via the Council of Australian University Librarians. No relevant disclosures. Not commissioned; externally peer reviewed.