Current state of the art MRI for the longitudinal assessment of cystic fibrosis

Abstract

Pulmonary MRI can now provide high‐resolution images that are sensitive to early disease and specific to inflammation in cystic fibrosis (CF) lung disease. With specificity and function limited via computed tomography (CT), there are significant advantages to MRI. Many of the modern MRI techniques can be performed throughout life, and can be employed to understand changes over time, in addition to quantification of treatment response. Proton density and T1/T2 contrast images can be obtained within a single breath‐hold, providing depiction of structural abnormalities and active inflammation. Modern radial and/or spiral ultrashort echo‐time (UTE) techniques rival CT in resolution for depiction and quantification of structure, for both airway and parenchymal abnormalities. Contrast perfusion MRI techniques are now utilized routinely to visualize changes in pulmonary and bronchial circulation that routinely occur in CF lung disease, and noncontrast techniques are moving closer to clinical translation. Functional information can be obtained from noncontrast proton images alone, using techniques such as Fourier decomposition. Hyperpolarized‐gas MRI, increasingly using 129Xe, is now becoming more widespread and has been demonstrated to have high sensitivity to early airway obstruction in CF via ventilation MRI. The sensitivity of 129Xe MRI promises future use in personalized medicine, management of early CF lung disease, and in future clinical trials. By combining structural and functional techniques, with or without hyperpolarized gases, regional structure–function relationships can be obtained, giving insight into the pathophysiology of disease and improved clinical management. This article reviews the modern MRI techniques that can routinely be employed for CF lung disease in nearly any large medical center. Level of Evidence: 4 Technical Efficacy Stage: 5 J. Magn. Reson. Imaging 2019.

Will Airway Gene Therapy for Cystic Fibrosis Improve Lung Function? New Imaging Technologies Can Help Us Find Out

“…This has made lung UTE MRI feasible for assessing CF structural lung disease, due to its ability to detect very subtle lung physiology, with scans acquired within a single breath hold. 63 Scoring systems can also be used for assessing lung structure through MRI, in a similar manner to CT. 64,65 As a result, UTE MRI can be used to quantify the extent and severity of lung abnormalities in very young patients with CF using a CF-specific scoring system, with a similar accuracy to CT and without ionizing radiation. 66,67 Nonetheless, despite these clear improvements in assessing lung structure through CT and MRI, methods that can provide functional information about the lung with high spatial and temporal resolution are required.…”

“…Most previous work has utilized 3 He, as it is easier to polarize than 129 Xe, and produces a higher signalto-noise ratio. However 129 Xe is cheaper and more readily available, 63 although it can be an anesthetic at high concentrations. The gases are administered immediately before MRI, using a breath hold to acquire images without respiratory motion.…”

“…Hyperpolarized MRI methods are robust and repeatable. 63 They have also been shown to have a higher diagnostic sensitivity than spirometry, LCI, and CT for detecting early stage CF lung disease. 69 Other studies have demonstrated that these techniques can be applied to unsedated infants and children, 70 suggesting that they should have high clinical utility in tracking CF disease progression.…”

Designing Clinical Trials for Anti-Inflammatory Therapies in Cystic Fibrosis

“…Pulmonary MRI can now provide high-resolution images that are sensitive to early disease and specific to inflammation in cystic fibrosis (CF) lung disease (Amin and Ratjen, 2008;Tiddens et al, 2015;Ciet et al, 2017;Woods et al, 2019). Unlike CT, MRI does not use ionizing radiation, and this is particularly advantageous in children and when scans need to be repeated within a relatively short time period (Amin and Ratjen, 2008;Tiddens et al, 2015).…”

“…Unlike CT, MRI does not use ionizing radiation, and this is particularly advantageous in children and when scans need to be repeated within a relatively short time period (Amin and Ratjen, 2008;Tiddens et al, 2015). MRI techniques can track changes in lung function longitudinally and quantify treatment response (Rayment et al, 2018;Santyr et al, 2019;Woods et al, 2019). Proton density and T 1 /T 2 contrast images can be obtained within a single breath-hold, providing a depiction of structural abnormalities and active inflammation.…”