Issue: 2015 > May > review

Non-cystic fibrosis bronchiectasis: clinical presentation, diagnosis and treatment, illustrated by data from a Dutch Teaching Hospital

J. Altenburg, K. Wortel, T.S. van der Werf, W.G. Boersma
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This review article describes the epidemiology, clinical presentation, diagnostic workup and treatment options in adult non-cystic fibrosis (non-CF) bronchiectasis (widening of mainly small and medium-sized bronchi as seen on chest computed tomography (CT) scan). We illustrate evidence from the literature with our own data retrieved from chart review, involving 236 adult patients with recurrent lower respiratory tract infections and high-resolution CT-proven non-CF bronchiectasis, who visited the outpatient clinic for respiratory diseases of a large Dutch teaching hospital between 2000 and 2010.
Non-CF bronchiectasis can be described as a final common pathway of a vicious cycle of excessive bronchial inflammation, bacterial colonisation and infection. Non-CF bronchiectasis may arise from several causes, headed by infection and immunodeficiency, and is clinically characterised by a chronic, productive cough and infectious exacerbations. Once non-CF bronchiectasis is diagnosed using high-resolution CT scanning, a protocol-driven work-up to identify the underlying cause is recommended. Non-medicinal treatment options are primarily directed at clearance of bronchial secretions, which can further be improved by inhalation of hyperosmolar agents. Antibiotic treatment of exacerbations is a cornerstone medicinal treatment in bronchiectasis management. Patients with frequent exacerbations can be considered for long-term low-dose macrolide treatment, supported by robust evidence. Inhaled antibiotics might be beneficial in selected patients colonised with Pseudomonas aeruginosa. Important developments in the last decade include the introduction of international guidelines and the proposal for a validated scoring system for disease severity. Bronchiectasis patients are encountered by physicians in diverse medical professions and the disease itself is still underdiagnosed. The authors aim to increase awareness of the condition and provide practical tools for diagnosis and treatment.