Respiratory specialist Dr Fiona Mosgrove explains the symptoms and pathophysiology of bronchiectasis in adults, and how to ensure it is diagnosed and managed appropriately
Key points
- Bronchiectasis may result from a prior respiratory infection such as pertussis or TB, and is also associated with COPD, asthma, RA, IBD, immunodeficiency and muco-ciliary disorders. However, half of patients have no identified cause
- The pathophysiology of bronchiectasis involves the following four factors, stemming from the underlying cause, each of which feeds into another: muco-ciliary dysfunction; neutrophilic inflammation; chronic infection; and structural airway damage
- Bronchiectasis in adults most commonly affects people over 40 and typically presents as chronic cough lasting more than eight weeks, with green or dark yellow sputum, or as recurrent chest infections requiring antibiotics
- An HRCT scan is required to diagnose bronchiectasis; a normal chest X-ray does not rule it out. Spirometry may help rule out other diagnoses
- The cornerstone of management is use of airway clearance techniques overseen by respiratory physiotherapists, along with treatment of the underlying cause
- Exacerbations should be treated promptly with antibiotics, with sputum culture to select the appropriate antibiotic
- ICSs should not routinely be part of bronchiectasis management, only for appropriate treatment of co-existing asthma or COPD
- The prognosis for patients with bronchiectasis is mixed and depends on frequency of exacerbations. While some are only mildly affected, those who experience frequent exacerbations have increased symptom burden and reduced life expectancy
Dr Fiona Mosgrove is a GPSI in respiratory medicine in Aberdeen and member of the Primary Care Respiratory Society education committee. Dr Mosgrove holds a master’s degree in respiratory medicine with a dissertation on bronchiectasis
How is bronchiectasis defined and what causes it?
Bronchiectasis in adults is a complex, chronic inflammatory condition affecting the airways of the respiratory system. It displays a high degree of heterogeneity in underlying aetiology and disease severity. Bronchiectasis is diagnosed in patients with relevant symptoms by the finding of dilated bronchi on high resolution computed tomography (HRCT).1
The pathophysiology of bronchiectasis was first described in the 1980s, as a vicious cycle.2 The cycle illustrates the sequential interaction between muco-ciliary dysfunction, neutrophilic inflammation, chronic infection and structural airway damage, with each factor leading to the next. Research published in the past 10 years has led to a new understanding, known as the vicious vortex, which demonstrates a more complex interaction between these four factors.3
Up to 50% of patients with bronchiectasis will have no underlying cause found for their disease. Bronchiectasis can occur as a complication of prior respiratory infection, such as measles or pertussis. It can also be associated with other conditions of the respiratory airways such as chronic obstructive pulmonary disease (COPD) and asthma. There is a known link with rheumatoid arthritis (RA) and also with inflammatory bowel disease (IBD), and some cases are caused by immunodeficiencies, such as human immunodeficiency virus (HIV) or muco-ciliary disorders such as primary ciliary dyskinesia (PCD).1
Patients can enter the vortex at any point, which helps to explain the varied underlying aetiology: it involves a spectrum of conditions. A patient with a ciliary disorder experiences ciliary dysfunction, leading to an increased susceptibility to infection, for example. A patient with an immunodeficiency may develop an infection that leads to persistent neutrophilic inflammation.
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