CFMR-lung: MR Imaging of Lung in the Follow-up Assessment of Cystic Fibrosis

Sponsor

University Hospital, Bordeaux (Other)

Overall Status

Active, not recruiting

CT.gov ID

NCT03357562

Collaborator

Ministry of Health, France (Other)

212

Enrollment

Locations

Arm

77.9

Anticipated Duration (Months)

17.7

Patients Per Site

0.2

Patients Per Site Per Month

Study Details

Study Description

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

Condition or Disease	Intervention/Treatment	Phase
Cystic Fibrosis	Device: lung MRI	N/A

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

Study Design

Study Type:

Interventional

Actual Enrollment :

212 participants

Allocation:

N/A

Intervention Model:

Single Group Assignment

Masking:

None (Open Label)

Primary Purpose:

Prevention

Official Title:

MR Imaging of Lung in the Follow-up Assessment of Cystic Fibrosis

Actual Study Start Date :

May 5, 2018

Anticipated Primary Completion Date :

Oct 31, 2024

Anticipated Study Completion Date :

Oct 31, 2024

Arms and Interventions

Arm	Intervention/Treatment
Experimental: lung MRI lung MRI without contrast injection	Device: lung MRI lung MRI without contrast injection

Arm

Intervention/Treatment

Experimental: lung MRI

lung MRI without contrast injection

Device: lung MRI

lung MRI without contrast injection

Outcome Measures

Primary Outcome Measures

Sensitivity of MRI to detect lung changes [Month 36]
deterioration or improvement measured by the Helbich-Bhalla scoring with CT as gold standard

Secondary Outcome Measures

Sensitivity of MRI to Helbich-Bhalla scoring change [Month 0 and Month 36]
Sensitivity of MRI to Helbich-Bhalla scoring change in various subgroups of patients according to age, centers and MR scan manufacturers, and new treatment drug use (Ivacaftor/lumicaftor: Orkambi Ø or Ivacaftor : Kalydeco Ø ) from CT and MR examinations
CT / MR concordance [Month 0 and Month 36]
Concordance between CT and MR in amplitude of Helbich-Bhalla scoring variations at M0 and M36
Sensitivity of the 3D-UTE MR sequence [Month 0 and Month 36]
Sensitivity of the 3D-UTE MR sequence alone to detect change in Helbich-Bhalla scoring as compared to CT performed at M0 and M36
Imaging quality of the 3D-UTE MR [Month 0, Month 12, Month 24 and Month 36]
using a likert scale
Correlation between a specific Helbich-Bhalla MR score and the amplitude of change [Month 0 and Month 36]
Correlation between a specific Helbich-Bhalla MR score with clinical and functional data, and concordance with the amplitude of change between M0 and M36
Accuracy of a lung MR protocol [Month 0 and Month 36]
Accuracy of a lung MR protocol including T1-weighted and T2-weighted sequences to diagnose allergic broncho-pulmonary aspergillosis (ABPA) in CF patients
Reproducibility in detecting lung structural abnormality [Month 0 and Month 36]
MR and CT reproducibility in detecting lung structural abnormality at the segmental level
Reproducibility in overall Helbich-Bhalla scoring [Month 0 and Month 36]
MR and CT reproducibility in overall Helbich-Bhalla scoring
Correlations between Helbich-Bhalla scoring and clinical questionnaire [Month 0 and Month 36]
Correlations between Helbich-Bhalla scoring measured with MRI and CT and clinical questionnaire
Correlations between Helbich-Bhalla scoring and exacerbation rate [Month 0 and Month 36]
Correlations between Helbich-Bhalla scoring measured with MRI and CT and exacerbation rate
Correlations between Helbich-Bhalla scoring and clinical pulmonary functional test [Month 0 and Month 36]
Correlations between Helbich-Bhalla scoring measured with MRI and CT and clinical pulmonary functional test

Eligibility Criteria

Criteria

Ages Eligible for Study:

8 Years and Older

Sexes Eligible for Study:

All

Accepts Healthy Volunteers:

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:

Contacts and Locations

Locations

	Site	City	Country	Postal Code
1	University Hospital of Bordeaux	Bordeaux	France	33000
2	University Hospital of Bordeaux	Bordeaux	France	33076
3	Hôpital Henri Mondor	Créteil	France	94010
4	CHRU de Grenoble	Grenoble	France	38043
5	CHRU de Lille	Lille	France	59000
6	Hopital Nord	Marseille	France	13385
7	Hopital Universitaire de la Timone	Marseille	France	13385
8	Fondation Lenval	Nice	France	06200
9	Hôpital Armand-Trousseau	Paris	France	75012
10	Hôpital Necker Enfant Malades	Paris	France	75015
11	Hôpital Foch	Suresnes	France	92150
12	CHRU Bretonneau-Tours	Tours	France	37044