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Bronchiectasis (brong-ke-EK-tah-sis) is the abnormal widening of the airways in the lungs usually caused by damage to the airway walls. This condition is characterised by a persistent cough with excess amounts of mucus and, often, airflow obstruction together with episodes of worsening symptoms.
In healthy individuals, glands in the lining of the airways make small amounts of mucus, which keeps the airways moist and traps inhaled dust, dirt and organisms. Usually, when mucus becomes excessive it is either coughed up and out, or swallowed. However, in people with bronchiectasis, the mucus pools where the airway is widened and makes the person prone to recurrent respiratory tract infections.
The symptoms, severity and disease course of bronchiectasis may vary. Chronic cough that brings up mucus and breathlessness are common symptoms, but some people may also experience fatigue, chest pain and weight loss (Goeminne & Dupont 2010). Bronchiectasis may cause long term disability, accelerated lung function loss and premature death in adults (Loebinger et al. 2009).
The underlying cause of bronchiectasis may vary between individuals, and is often undefined even after clinical investigation (King et al. 2006). The known causes of bronchiectasis include cystic fibrosis, reduced immune functioning, severe pneumonia, abnormal function of cells that line the airways, and a wide variety of other conditions. In some cases, no identified cause can be established.
Cystic fibrosis (CF) is a hereditary disease in which mucus from glands is thicker and stickier than normal, affecting the lungs and other organs. Most cases of bronchiectasis in adults are not associated with cystic fibrosis (non-CF bronchiectasis).
While there is consensus that cigarette smoking and passive smoking exacerbate respiratory conditions, a definitive causal link between smoking and bronchiectasis has not been shown.
Bronchiectasis can affect anyone at any age, and in any socioeconomic group, but the disease occurs much more commonly in rural and remote Indigenous communities and in less affluent communities (Chang et al. 2003; Karadag et al. 2004; Singleton et al. 2000; Twiss et al. 2005). This may be due to higher exposure to smoke from heating and cooking, limited access to quality health services, non-adherence to medications and inadequate medical follow-up (Chang et al. 2003).
There is little information available on the overall Australian prevalence or incidence of bronchiectasis.
Based on a study of hospital medical records, the estimated prevalence of bronchiectasis in Central Australian Indigenous children was 15 per 1,000 population (Chang et al. 2003). In the same study, no cases of bronchiectasis among non-Indigenous children were found, even though two-thirds of the population the hospital served were non-Indigenous.
Managing bronchiectasis effectively can require a broad range of healthcare providers from primary healthcare, hospital care through to palliative care for those with advanced disease. As such, it is ideally managed in the community with primary healthcare providers acting as coordinators of care (McGuire 2012). However, despite the important role it plays, activities of the Australian primary healthcare system are currently not nationally monitored. The Bettering the Evaluation and Care of Health (BEACH) survey of general practitioners provides a window into general practice activities but does not provide reliable information for relatively uncommon conditions such as bronchiectasis.
Management of this condition is complex because of the variety of underlying causes. Clinical decisions around the management of the condition are made based on individual presentations. Treatment may include physiotherapy, use of medicines (particularly to control infections), regular influenza vaccinations and, where appropriate, surgery (Chang et al 2010).
Some people with bronchiectasis require treatment in hospital, particularly for the management of severe disease exacerbations. Aside from the severity of the exacerbations, other factors that affect hospitalisation rates include improved diagnosis, changes in clinical management of the condition, and admission practices.
The Australian Institute of Health and Welfare (AIHW) National Hospital Morbidity Database (NHMD) contains information about admitted-patient services provided in Australia.
The AIHW NHMD 2010–11 showed:
The hospitalisation rate for bronchiectasis as a principal diagnosis increased steadily from 1998–99 to 2011–12 (from 14 to 21 per 100,000 population respectively). The rate increased for both females (9 per 100,000 population to 14 per 100,000 population) and males (5 per 100,000 population to 7 per 100,000 population) (Figure 1). It is not possible to determine from the source data to what extent this increase is due to an increase in the prevalence of the condition or in other factors affecting hospitalisation rates.
Source: AIHW National Hospital Morbidity Database.
In 2010–11, for the 9,018 hospitalisations where bronchiectasis was an additional diagnosis, chronic obstructive pulmonary disease (COPD) (19%), cystic fibrosis (19%) and pneumonia (11%) were the three most common principal diagnoses. In the younger age groups (0–4 to 45–49), bronchiectasis as an additional diagnosis was more often related to underlying cystic fibrosis, while in older age groups, it was more often associated with underlying COPD and to a lesser extent, pneumonia (Figure 2).
In 2011, there were 745 deaths where bronchiectasis was recorded as either the underlying (314) or an associated (431) cause of death (ABS 2013).
Australian Institute of Health and Welfare 2010. Asthma, chronic obstructive pulmonary disease and other respiratory diseases in Australia. Cat. no. ACM 20. Canberra. AIHW.
Source: AIHW National Hospital Morbidity Database. ICD-10-AM codes included are: Cystic fibrosis E84; COPD J40–J44; Pneumonia J12–J18.
ABS (Australian Bureau of Statistics) 2013. 3303.0 Causes of Death, Australia, 2011. Table 10.2 Multiple causes of death, All causes, Number of deaths by underlying cause and multiple cause mentions, 2011. Viewed on 9 July 2013, < Multiple causes of death, All causes, Number of deaths by underlying cause and multiple cause mentions, 2011>.
Chang A, Masel J, Boyce NC, Wheaton G & Torzillo P 2003. Non-CF bronchiectasis: clinical and HRCT evaluation. Pediatr Pulmonol 35:477–83.
Chang A, Bell S, Byrnes A, Grimwood K Holmes, P, King P et al. 2010. Chronic suppuratives lung disease and bronchiectasis in children and adults in Australia and New Zealand. Position statement from the Thoracic Society of Australia and New Zealand and the Australian Lung Foundation. Medical Journal of Australia 193:356–65.
Goeminne P & Dupont L 2010. Non-cystic fibrosis bronchiectasis: diagnosis and management in 21st century. Postgraduate Medicine Journal 86:493–501.
Karadag B, Karakoc F, Ersu R, Kut A, Bakac S & Dagli E 2005. Non-Cystic-Fibrosis Bronchiectasis in children: A persisting problem in developing countries. Respiration 72:223–8.
King P, Holdsworth S, Freezer N, Villanueva E & Holmes P 2006. Characterisation of the onset and presenting clinical features of adult bronchiectasis. Respiratory Medicine 100:2183–89.
Loebinger M, Wells A, Hansell, D, Chinyanganya N, Dayaraj, A, Maister M et al. 2009. Mortality in bronchiectasis: a long-term study assessing the factors influencing survival. European Respiratory Journal 34:843–9.
McGuire G 2012. Bronchiectasis. A guide for primary care. Australian Family Physician 41: 842–50.
NCCH (National Centre for Classification in Health) 2010. The International Statistical Classification of Disease and Related Health Problems, Tenth Revision, Australian Modification (ICD-10-AM). 7th edn. Tabular list of diseases and Alphabetic index of diseases. Sydney: NCCH, Faculty of Health Sciences, The University of Sydney.
Singleton R, Morris A, Redding G, Poll J, Holck P, Martinez P et al. Bronchiectasis in Alaska native children: Causes and clinical courses. Pediatric Pulmonology 29:182–7.
Twiss, J, Metcalfe R, Edwards E & Byrnes C. 2005. New Zealand national incidence of bronchiectasis “too high” for a developed country. Archives of Disease in Childhood 90:737–40.