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

Cystic Fibrosis Clinical Trial

— CFMR-lung  

Official title:

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

Clinical Trial Details — Status: Active, not recruiting

Administrative data

• NCT number: NCT03357562
• Other study ID #: CHUBX 2016/28
• Status: Active, not recruiting
• Phase: N/A
• Start date: May 5, 2018
• Est. completion date: October 31, 2024

Study information

• Verified date: May 2024
• Source: University Hospital, Bordeaux
• Contact: n/a
• Is FDA regulated: No
• Study type: Interventional

Clinical Trial 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

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

Recruitment information / eligibility

• Status: Active, not recruiting
• Enrollment: 212
• Est. completion date: October 31, 2024
• Est. primary completion date: October 31, 2024
• Accepts healthy volunteers: No
• Gender: All
• Age group: 8 Years and older

Eligibility

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:

Related Conditions & MeSH terms

• Cystic Fibrosis
• Fibrosis

Intervention

Device:
• lung MRI
lung MRI without contrast injection

Locations

Country	Name	City	State
France	University Hospital of Bordeaux	Bordeaux
France	University Hospital of Bordeaux	Bordeaux
France	Hôpital Henri Mondor	Créteil
France	CHRU de Grenoble	Grenoble
France	CHRU de Lille	Lille
France	Hopital Nord	Marseille
France	Hopital Universitaire de la Timone	Marseille
France	Fondation Lenval	Nice
France	Hôpital Armand-Trousseau	Paris
France	Hôpital Necker Enfant Malades	Paris
France	Hôpital Foch	Suresnes
France	CHRU Bretonneau-Tours	Tours

Sponsors (2)

Lead Sponsor	Collaborator
University Hospital, Bordeaux	Ministry of Health, France

Country where clinical trial is conducted

France, 

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	Sensitivity of MRI to detect lung changes	deterioration or improvement measured by the Helbich-Bhalla scoring with CT as gold standard	Month 36
Secondary	Sensitivity of MRI to Helbich-Bhalla scoring change	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	Month 0 and Month 36
Secondary	CT / MR concordance	Concordance between CT and MR in amplitude of Helbich-Bhalla scoring variations at M0 and M36	Month 0 and Month 36
Secondary	Sensitivity of the 3D-UTE MR sequence	Sensitivity of the 3D-UTE MR sequence alone to detect change in Helbich-Bhalla scoring as compared to CT performed at M0 and M36	Month 0 and Month 36
Secondary	Imaging quality of the 3D-UTE MR	using a likert scale	Month 0, Month 12, Month 24 and Month 36
Secondary	Correlation between a specific Helbich-Bhalla MR score and the amplitude of change	Correlation between a specific Helbich-Bhalla MR score with clinical and functional data, and concordance with the amplitude of change between M0 and M36	Month 0 and Month 36
Secondary	Accuracy of a lung MR protocol	Accuracy of a lung MR protocol including T1-weighted and T2-weighted sequences to diagnose allergic broncho-pulmonary aspergillosis (ABPA) in CF patients	Month 0 and Month 36
Secondary	Reproducibility in detecting lung structural abnormality	MR and CT reproducibility in detecting lung structural abnormality at the segmental level	Month 0 and Month 36
Secondary	Reproducibility in overall Helbich-Bhalla scoring	MR and CT reproducibility in overall Helbich-Bhalla scoring	Month 0 and Month 36
Secondary	Correlations between Helbich-Bhalla scoring and clinical questionnaire	Correlations between Helbich-Bhalla scoring measured with MRI and CT and clinical questionnaire	Month 0 and Month 36
Secondary	Correlations between Helbich-Bhalla scoring and exacerbation rate	Correlations between Helbich-Bhalla scoring measured with MRI and CT and exacerbation rate	Month 0 and Month 36
Secondary	Correlations between Helbich-Bhalla scoring and clinical pulmonary functional test	Correlations between Helbich-Bhalla scoring measured with MRI and CT and clinical pulmonary functional test	Month 0 and Month 36