Lung imaging in cystic fibrosis

Summary Management of CF patients currently relies on clinical status, lung function tests, sputum cultures and scoring systems based on chest X-ray (CXR). None of these tests give adequate information about regional lung function or are sensitive enough to demonstrate subtle changes that may aid in assessing early lung disease status, planning therapy or evaluating response to treatment. There is increasing evidence from recent studies with CT and MRI that current routine measures of lung physiology, (spirometry) and structure (CXR), may not accurately reflect disease progression nor identify early stages of lung disease, often remaining within a normal range even when significant and irreversible pulmonary damage has already occurred. Thus accurate, non-invasive, regional methods of diagnosis and follow up of CF patients based upon imaging endpoints are highly desirable. In this paper, state of the art methods of imaging signs of lung disease in the CF lung are reviewed with discussions on the technical advances in CT, proton and hyperpolarised gas MRI with example images from groups active in the area of CF lung imaging.