Bronchial artery embolisation in cystic fibrosis patients

Cystic fibrosis (CF) is a recessive genetic disease characterised by impaired muco-ciliary clearance. Although the disease affects multiple organs, 85% of mortality results from lung pathology relating to persistent recurrent infections, an exaggerated inflammatory response and progressive airways obstruction which ultimately leads to respiratory failure. Haemoptysis is a common symptom of CF with reported rates of up to 9% over a fiveyear period. The pathophysiology of haemoptysis in CF primarily relates to erosion of newly formed abnormal bronchial arteries which may be thin walled or tortuous in areas of bronchiectasis secondary to chronic infection. These vessels are prone to injury or rupture during acute infective exacerbations leading to haemoptysis. A further precipitating factor may be coagulopathy secondary to Vitamin K deficiency due to the hepatic effects of CF. Massive haemoptysis (defined as haemoptysis >240ml/day) is seen less commonly in CF patients than recurrent nonmassive haemoptysis, but when present can be a life-threatening condition that requires urgent treatment to prevent death from asphyxiation or exsanguination. Recurrent non-massive haemoptysis carries a significant morbidity in terms of precluding effective airway clearance and may be a predictor of future massive haemoptysis and excess mortality. It is therefore a concerning symptom that requires further assessment. The various approaches to guide treatment include chest radiography, CT examination, bronchoscopy and respiratory function tests. In contrast to many of the other causes of haemoptysis, the CF population is generally younger, which may have a logistical impact on development of an embolisation service.