Respirology | 2019
Moving forward: Bronchiectasis and chronic suppurative lung disease in children and adults in the 21st century
Abstract
This has been an excellent series discussing the aetiology, investigation and management of paediatric and adult bronchiectasis and is timely, with a growth of interest in bronchiectasis. This is reflected in a surge of randomized controlled trials in bronchiectasis in the last 10 years, increasing from 91 randomized controlled trials in 2008 to 252 by July 2019. The reviews highlight important insights learned from treating children with bronchiectasis. The spectrum from protracted bacterial bronchitis to frank radiological bronchiectasis fits with the vicious cycle hypothesis. However, as a proportion of frank bronchiectasis in children is reversible, there is a challenge to the concept that bronchiectasis is irreversible in adults, which argues for targeted early treatment in bronchiectasis to avert disease progression and perhaps regress the degree of bronchiectasis. This has not been explored to date but represents an interesting hypothesis for the future. Bush and Floto highlighted that current testing fails to identify a cause for bronchiectasis in many cases. With the more widespread use of whole-genome sequencing, it is hoped that we will identify a cause for bronchiectasis in most cases, informing the development of future therapeutic targets. Identifying the cause of bronchiectasis is important as this may guide future management. This is reinforced in the British Thoracic 2019 Guidelines in Bronchiectasis, highlighting that all patients should be investigated for potentially treatable causes of bronchiectasis. Quint and Smith highlighted the prevalence of bronchiectasis in children, particularly the link with social disadvantage. In adults, there is an increased prevalence in middle-aged and elderly cohorts. Understanding this epidemiology will help target prevention and treatment plans. In socially disadvantaged populations, there needs to be international efforts to promote quality standards in bronchiectasis, such as education in chest clearance techniques, vaccinations and prompt treatment of respiratory tract infections. The distinct lack of vaccine trials was highlighted by O’Grady et al., and further bespoke trials are needed. Targeting those in high-risk populations would be an excellent strategy to assess the safety and efficacy of vaccines. Unless the inequity of treatment issues is addressed, these disadvantaged groups will continue to have more severe bronchiectasis, leading to a shortened life expectancy. O’Neill et al. described the importance of individualized, age-appropriate chest clearance techniques taught by a respiratory physiotherapist. We need to address why there is poor adherence and develop strategies to promote chest clearance, so patients adhere to daily therapy. Mucoactive treatments may be additive to airway clearance techniques, but the evidence base is weak; further randomized controlled trials are needed. There is a need for bespoke pulmonary rehabilitation programmes for bronchiectasis targeting those with breathlessness affecting their activities of daily living. In contrast to patients with cystic fibrosis, it appears that there are no major cross-infection safety issues, but this will need to be monitored. Bell et al. discussed the challenge of international consensus on the definition of an exacerbation. Although there has been an international consensus on the definition of an exacerbation for use in clinical trials, more work is needed to assess feasibility in both clinical practice and the clinical trial setting. For more advanced disease, Bell et al. updated the evidence base for long-term therapies, particularly long-term macrolides and other antibiotics. There is a need for licensed therapies, both anti-inflammatory and anti-infective, in bronchiectasis, and further research is needed on whom to treat, what agent/s to use and duration of therapy. Regan and Hill highlighted potential future novel therapies, but international randomized controlled trials are needed before such therapies would be considered safe for standard treatment. In middle-aged and elderly people, there needs to be increased attention to co-morbid disease, which can affect the progression of bronchiectasis. Maglione et al. described specific management in children and adults with bronchiectasis who have comorbid airways disease, inflammatory arthritis and inflammatory bowel disease. It would be helpful for future randomized controlled trials to investigate the specific management in these groups of patients to determine optimum treatment(s) for both bronchiectasis and the associated condition. Studies have also highlighted increased vascular disease in patients with bronchiectasis related to the severity of bronchiectasis. Future randomized controlled trials should address secondary prevention in patients with more advanced bronchiectasis. Navaratnam et al. highlighted the need for multidisciplinary teams to guide long-term management of