Bronchiectasis

What is bronchiectasis?

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.

Symptoms of bronchiectasis

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 [1]. Bronchiectasis may cause long term disability, accelerated lung function loss and premature death in adults [2].

Causes of bronchiectasis

The underlying cause of bronchiectasis may vary between individuals, and is often undefined even after clinical investigation [3]. 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. 

Who gets bronchiectasis?

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 [4,5,6,7]. 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 [1].

There is little information available on the prevalence or incidence of bronchiectasis in Australia. The Australian Bronchiectasis Registry, which opened in 2016, is a non-commercial research project that has been initiated by a group of Australian Respiratory Specialist doctors together with The Lung Foundation Australia. The main aims of this registry are to identify and collect health information from patients with non-Cystic Fibrosis (non-CF) Bronchiectasis for doctors to research the causes and to improve treatments. For more information visit The Lung Foundation of Australia.

Bronchiectasis does act differently in women than in men, particularly in the areas of prevalence, incidence, causative organisms and etiology. Specifically, cystic fibrosis (CF) and non-CF bronchiectasis follow a more severe course and appear more frequently in women [8].

References

  1. Goeminne P & Dupont L 2010. Non-cystic fibrosis bronchiectasis: diagnosis and management in 21st century. Postgraduate Medicine Journal 86:493–501.
  2. 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.
  3. 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.
  4. Chang A, Masel J, Boyce NC, Wheaton G & Torzillo P 2003. Non-CF bronchiectasis: clinical and HRCT evaluation. Pediatr Pulmonol 35:477–83.
  5. 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.
  6. 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.
  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.
  8. Morrissey B.M, & Harper, R.W (2004). Bronchiectasis: sex and gender considerations. Clinics in Chest Medicine 25: 361─372.