Assessment of lung impairment in patients with cystic fibrosis : Novel magnetic resonance imaging methods

Abstract

The differentiated assessment of respiratory mechanics, gas exchange and pulmonary circulation, as well as structural impairment of the lung are essential for the treatment of patients with cystic fibrosis (CF). Clinical lung function measurements are often not sufficiently specific and are often difficult to perform. The standard procedures for pulmonary imaging are chest X‑ray and computed tomography (CT) for assessing lung morphology. In more recent studies, an increasing number of centers are using magnetic resonance imaging (MRI) to assess lung structure and function. However, functional imaging is currently limited to specialized centers. In patients with CF, studies showed that MRI with hyperpolarized gases and Fourier decomposition/matrix pencil MRI (FD/MP-MRI) are feasible for assessing pulmonary ventilation. For pulmonary perfusion, dynamic contrast-enhanced MRI (DCE-MRI) or contrast-free methods, e.g., FD-MRI, can be used. Functional MRI provides more accurate insight into the pathophysiology of pulmonary function at the regional level. Advantages of MRI over X‑ray are its lack of ionizing radiation, the large number of lung function parameters that can be extracted using different contrast mechanisms, and ability to be used repeatedly over time. Early assessment of lung function impairment is needed as the structural changes usually occur later in the course of the disease. However, sufficient experience in clinical application exist only for certain functional lung MRI procedures. Clinical application of the aforementioned techniques, except for DCE-MRI, should be restricted to scientific studies.