Table 1.

Summary of respiratory imaging techniques used in patients with Cystic Fibrosis

Method	Technique	Role	Advantages	Disadvantages	Ref
CT	HRCT	• Lung anatomy/morphology assessment • Disease progression evaluation • Treatment response evaluation	+ quick + easy to apply + well established in clinical practice + high spatial resolution	- ionizing radiation method - lack of pulmonary function assessment	[16–19]
CT	CV-HRCT	• Lung anatomy/morphology assessment in infants • Disease progression evaluation • Treatment response evaluation	+ assess lung changes in infants + high spatial resolution	- ionizing radiation method - lack of pulmonary function assessment	[20–24]
MRI	IV CE-MRI	• Assessment of pulmonary vascular bed perfusion • Lung structure evaluation	+ Radiation free method + pulmonary function assessment (perfusion)	- not applicable for all patients (metal parts in body, pacemaker etc.) - low spatial resolution due to low proton density of lungs - GD contrast can cause allergies	[27, 29–31]
MRI	3He MRI	• Assessment of ventilation and diffusion functional imaging • Disease progression evaluation • Treatment response evaluation	+ radiation free method + sensitive track early progression of the disease, early treatment response + quantitative information about function (ventilation defect, apparent diffusion coefficient)	- not applicable for all patients (metal parts in body, pacemaker etc.) - low spatial resolution due to low proton density of lungs - currently not applicable to infants due to complicated protocol	[34–44]
MRI	129Xe MRI	• Assessment of ventilation, diffusion and perfusion possible during one imaging protocol • Disease progression evaluation • Treatment response evaluation	+ radiation free method + sensitive- track early progression of the disease, early treatment response + in-vivo observation of gas dissolution into barrier and RBCs + quantitative information about function (ventilation defect, apparent diffusion coefficient, gas-transfer defect)	- not applicable for all patients (metal parts in body, pacemaker etc.) - low spatial resolution due to low proton density of lungs - up to date not applicable to infants due to complicated protocol - potentially hazardous due to Xe anaesthetic properties	[46–50, 52]
MRI	Non CE-MRI: ASL	• Assessment of perfusion • Disease progression evaluation	+ radiation free method + pulmonary function assessment (perfusion) + quantitative information (perfusion heterogeneity)	- not applicable for all patients (metal parts in body, pacemaker etc.) - low spatial resolution due to low proton density of lungs	[55, 58]
MRI	Non CE-MRI: FD MRI	• Assessment of ventilation and perfusion • Disease progression evaluation	+ radiation free method + pulmonary function assessment (perfusion and ventilation) + quantitative information (perfusion homogeneity + results comparable to single photon emission CT and CE-MRI but without exogenous contrast	- not applicable for all patients (metal parts in body, pacemaker etc.) - low spatial resolution due to low proton density of lungs	[60, 61]
MRI	Non CE-MRI: nT1	• Assessment of perfusion • Disease progression evaluation	+ radiation free method + pulmonary function assessment (perfusion and ventilation) + quantitative information (dissolution phase homogeneity)	- not applicable for all patients (metal parts in body, pacemaker etc.) - low spatial resolution due to low proton density of lungs	[62, 63]
MRI	Non CE-MRI: DWI	• Assessment of diffusion changes to localize mucus plugs	+ radiation free method + diffusion changes- localization of areas with elevated diffusion called hotspots	- not applicable for all patients (metal parts in body, pacemaker etc.) - low spatial resolution due to low proton density of lungs	[65]
Synchrotron based X-Ray	Phase contrast x-ray imaging	• Assessment of structural and functional changes in a microscale	+ very high spatial and temporal resolution of images + cimaging function and morphology in microscale	- ionizing radiation method- not applicable for humans	[66, 68–70]