Historically, non-cystic fibrosis (non-CF) bronchiectasis has attracted few advocates from the medical, pharmaceutical or broader community and has therefore been considered an orphan disease. More recent initiatives have substantially increased interest and research in non-CF bronchiectasis, leading to a strengthening evidence base for therapies and the development of a national bronchiectasis registry (https://lungfoundation.com.au/research/our-research/bronchiectasis). Guidance for health professionals who are providing care to children or adults with bronchiectasis is available in the form of Australian and international guidelines and other resources derived from them (Box 1).2,3 Seeking and confirming a diagnosis of bronchiectasis allows the provision of disease-appropriate treatment, with major potential benefit in both disease prognosis and quality of life for those with this condition.
Incidence, morbidity and mortality
Globally, there has been a substantial increase in the recognition and diagnosis of bronchiectasis. Prevalence data for bronchiectasis in Australia are scant, yet this condition may affect up to 1.5% of children aged 15 years and under who are referred to respiratory physicians. Population estimates vary from four to 1470 for every 100,000 children in Australia and New Zealand.4
In 2015, bronchiectasis was diagnosed in more than 17,000 hospitalisations in Australia and hospitalisation rates for females were double male rates. Hospital length of stay for bronchiectasis is typically double the duration of all other hospitalisations in Australia and has significant associated increases in cost estimates.5 Bronchiectasis as a primary or co-diagnosis tends to prolong hospital length of stay, increases the likelihood of admission to the intensive care unit and mortality in those with coexistent COPD and is associated with an increased risk of a first cardiovascular event.6,7 Review of observational studies has suggested that bronchiectasis may coexist with and complicate COPD (in 54% of cases) and uncontrolled severe asthma (in 34%).8,9
CF has an incidence of one in 2000 live births in Australia, and children with the condition are usually affected by bronchiectasis. A family history of bronchiectasis is therefore important, as CF, primary ciliary dyskinesia and Kartagener’s syndrome share an autosomal recessive inheritance pattern.
Globally, Indigenous populations, and particularly Indigenous Australian children, are at significantly increased risk of bronchiectasis and chronic suppurative lung disease and share a disproportionately large burden of disease and number of associated hospital admissions.10,11 Importantly for these children, appropriate early management may make chronic suppurative lung disease largely preventable. A further important risk group is patients with established lung disease; bronchiectasis has been identified in up to 50% of patients with COPD.12,13
Quality of life is substantially impaired in patients with bronchiectasis and is further adversely affected during exacerbations. Mortality among bronchiectasis cohorts has been identified as being as high as 16 to 30% over a decade, mainly affecting the elderly.14
Pathologically, bronchiectasis is a heterogeneous condition in which follicular, saccular and atelectatic histological patterns have been described.15 This pathological diversity may suggest that pathological bronchiectatic airway dilatation may be the final common pathway of several infective and immunological airway insults. The follicular pattern appears most frequently, usually involving the smaller airways. In the mildest forms, subepithelial lymphoid aggregations and oedema are the only findings.