A twenty-nine year old female non-smoker presents to her general practitioner with a cough, producing large volumes of purulent sputum. She has had several of these episodes over the years, but believes this is due to working in a nursery, as many of the children have "coughs and colds."
A full history should be ascertained, addressing areas such as past medical history, drug history and recent foreign travel. A respiratory history should be taken, paying particular attention to the following symptoms:
In addition to the chronic cough with the production of purulent sputum, the patient also admitted episodes of pleuritic chest pain and an episode of haemoptysis. Auscultation of her chest revealed crackles and wheeze. As she had already been on courses of antibiotics, the GP sent off a sputum culture and referred her to a chest physician.
The role of investigations in bronchiectasis are to ascertain the cause and assess the severity of the disease. The following investigations are considered appropriate according to the British Thoracic Society:
The patient's sputum culture grew Pseudomonas aeruginosa. Given this unusual organism, the chest physician requires a high-resolution CT scan, which demonstrated areas of bronchial wall thickening and dilatation, giving a diagnosis of bronchiectasis. Other investigations, such as immunoglobulins and CFTR status, were normal.
Bronchiectasis can be defined as an irreversible dilatation of the bronchi caused by destruction of the muscular and elastic tissue of the bronchial walls [Rubin R, Strayer DS. Rubin's Pathology]. It was once much more common, usually resulting from childhood respiratory infections, but vaccination and antibiotic therapy have reduced the prevalence.
Bronchiectasis has several different causes, which includes the following:
The pathogenesis of bronchiectasis is complex and several different mechanisms have been proposed. A "vicious circle" of events is most likely to take place, involving damage to the bronchial epithelium which allows colonisation by bacteria leading to impaired ciliary clearance and subsequent inflammation. Some of the bacteria known to do this are Haemophilus influenzae, Streptococcus pneumoniae and Pseudomonas aeruginosa.
Due to the prolonged and repeated bouts of inflammation, there is weakening of the bronchial walls, due to proteolytic enzymes and reactive oxygen species (ROS) released from neutrophils, leading to dilatation.
On gross inspection, the bronchi are dilated and have thickened walls, with collapse of the distal lung. The lumen frequently contain think, purulent secretions. Histologically, there is severe inflammation, leading to destruction of all layers of the bronchial walls. The bronchial arteries are enlarged to meet the additional vascular requirements to sustain the inflammation and fibrosis.
Complications include the development of a lung abscess and empyema.
Patients with bronchiectasis need to be managed by a multi-disciplinary team involving communication between the general practitioner, chest physician, microbiologist, physiotherapy and primary care nurses. The management according to the British Thoracic Society includes:
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The case scenario was adapted from ten Hacken NHT, van der Molen T, Easily missed? Bronchiectasis. BMJ 2010;341:c2766
Figure 2: taken from The Newcastle upon Tyne Hospitals. Cardiothoracic Services [online]. 15th March 2012. Available from URL: http://www.newcastle-hospitals.org.uk/services/cardiothoracic_services_bronchiectasis.aspx
Figure 3: taken from Pitney AC, Callahan CW, Reuss L. Reversal of bronchiectasis caused by chronic aspiration in cri du chat syndrome. Images in paediatric medicine 2001;84: 413-414
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