mr imaging of lung in the follow-up assessment of cystic fibrosis

Conditions: Cystic Fibrosis

Summary: the aim of the study is to assess the diagnostic sensitivity of mri to detect changes in helbich-bhalla scoring over time in patients with cystic fibrosis

cystic fibrosis (cf) is caused by the cystic fibrosis transmembrane conductance regulator (cftr) gene mutation and represents one of the most frequent and lethal inherited disease in caucasian. however, thanks to better treatments that slow down the progression of pulmonary disease, the median life expectancy has reached 41 years and there are nowadays more cf patients older than 18-year-old than younger. chronic lung disease is the main manifestation and represents more than 90% of cf morbidity and mortality. however, there is a need for biomarkers more sensitive than clinical and functional findings for a personalized management of patients. computed tomography (ct), owing to its high spatial resolution and contrast, is the standard of reference in imaging for depicting lung structural alterations. but ct is an ionizing technique, rising concern in cancer risk associated to cumulated radiation dose. to date, magnetic resonance imaging (mri) is a radiation-free technique which has been demonstrated to add meaningful functional information that cannot be reached using ct. recent advances in 3-dimensional ultra-short echo time (3d-ute) imaging have been shown promising to improve lung mr imaging quality. a clear delineation between airway wall and lumen was obtained, thanks to submillimeter voxel size, enabling readers to estimate both bronchial thickening and dilatation with very good concordance with ct, independently from the magnitude of score. the combination of pulse sequence may rather benefit from the potential of mri to get more complete insight into inflammatory processes by combining several contrasts, as compared to other ionizing methods. novel mr methods have been shown promising in assessing lung changes with high resolution and therefore could be proposed instead of ct for radiation- free repeated, life-long follow-up

Eligibility criteria:
inclusion criteria: * male or female children (age ≥ 8 y.o) and adult patient with a diagnosis of cystic fibrosis provided by genetic and swear test older than 8 years. subgroups of patients will be defined according to: * age: younger or older 18y.o. we expect around 50% in different subgroups. in case of, we reach 50% in one of these groups, patient recruitment will continue for both groups until the expected number of patients in the study is reached. * brand name of magnet: siemens, general electric or philips * new drugs use: association ivacaftor/lumicaftor (orkambiø) or ivacaftor only (kalydeco ø) ) we expect approximately 20% to 50% of patients treated * informed consent provided to the patient or/and to legal representative for adults and to parents for the children * patient concerned by articles l 1121-6, l 1121-7, and l 1121-8 (persons deprived of their liberty by a judicial or administrative decision, minors, persons of legal age who are the object of a legal protection measure or unable to express their consent) if the expected benefit for such persons justifies the foreseeable risk incurred exclusion criteria: * patients without any social security or health insurance * pregnant women * patients with previous pulmonary transplantation or planned for transplantation in the year following inclusion * mri contraindications:

Keywords: cystic fibrosis ct mri radiation dose contrast agent