This paper systematically reviewed the literature reporting the validity and reliability of hyperpolarised gas MRI as a marker of lung health in cystic fibrosis (CF). MEDLINE, EMBASE and grey literature were searched for studies assessing the measurement properties of hyperpolarised helium-3 or xenon-129 MRI. The COSMIN risk of bias tool was used to critically appraise eligible studies. Findings show hyperpolarised gas MRI was able to detect structural and functional abnormalities in the lungs, detect response to treatments, and is more sensitive than FEV in detecting ventilation defects in CF patients. There was moderately robust evidence for construct validity of hyperpolarised gas MRI, although evidence for other types of validity is currently low. Nonetheless, high quality studies concluded that hyperpolarised gas MRI is a reliable tool and test results are reproducible in CF patients. Hyperpolarised gas MRI is a promising tool for detecting early CF pulmonary disease and for longitudinal monitoring of CF.
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