Clinical trial NCT03357562: MR Imaging of Lung in the Follow-up Assessment of Cystic Fibrosis
Official title: MR Imaging of Lung in the Follow-up Assessment of Cystic Fibrosis
Study type: INTERVENTIONAL
Overall status: COMPLETED
Phase: NA
Conditions: Cystic Fibrosis
Keywords: Cystic fibrosis
CT
MRI
radiation dose
contrast agent
Lead sponsor: University Hospital, Bordeaux
Sponsor class: OTHER
Collaborators: name: Ministry of Health, France
First posted: 2017-11-30
Enrollment: 212
Sex: ALL
Minimum age (years): 8
Standard ages: CHILD
ADULT
OLDER_ADULT
Accepts healthy volunteers: no
Allocation: NA
Intervention model: SINGLE_GROUP
Primary purpose: PREVENTION

Brief 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

Detailed description: 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:

Primary outcomes:
measure: Sensitivity of MRI to detect lung changes
timeFrame: Month 36
Secondary outcomes:
measure: Sensitivity of MRI to Helbich-Bhalla scoring change
timeFrame: Month 0 and Month 36
measure: CT / MR concordance
timeFrame: Month 0 and Month 36
measure: Sensitivity of the 3D-UTE MR sequence
timeFrame: Month 0 and Month 36
measure: Imaging quality of the 3D-UTE MR
timeFrame: Month 0, Month 12, Month 24 and Month 36
measure: Correlation between a specific Helbich-Bhalla MR score and the amplitude of change
timeFrame: Month 0 and Month 36
measure: Accuracy of a lung MR protocol
timeFrame: Month 0 and Month 36
measure: Reproducibility in detecting lung structural abnormality
timeFrame: Month 0 and Month 36
measure: Reproducibility in overall Helbich-Bhalla scoring
timeFrame: Month 0 and Month 36
measure: Correlations between Helbich-Bhalla scoring and clinical questionnaire
timeFrame: Month 0 and Month 36
measure: Correlations between Helbich-Bhalla scoring and exacerbation rate
timeFrame: Month 0 and Month 36
measure: Correlations between Helbich-Bhalla scoring and clinical pulmonary functional test
timeFrame: Month 0 and Month 36
Other outcomes:
Locations:
city: Bordeaux
country: France
facility: University Hospital of Bordeaux
city: Bordeaux
country: France
facility: University Hospital of Bordeaux
city: Créteil
country: France
facility: Hôpital Henri Mondor
city: Grenoble
country: France
facility: CHRU de Grenoble
city: Lille
country: France
facility: CHRU de Lille
city: Marseille
country: France
facility: Hopital Nord
city: Marseille
country: France
facility: Hopital Universitaire de la Timone
city: Nice
country: France
facility: Fondation Lenval
city: Paris
country: France
facility: Hôpital Armand-Trousseau
city: Paris
country: France
facility: Hôpital Necker Enfant Malades
city: Tours
country: France
facility: CHRU Bretonneau-Tours
Study officials:
References: