A Prospective Monocentric Study to Assess the Concordance of Lung MRI Compared to Chest CT Scan to Assess the Extent and Severity of Bronchial and Parenchymal Pulmonary Lesions in Adult Patients With Primary Immune Deficiency (PID) .

Study Overview
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Brief Summary
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Currently, there is no official recommendations for the respiratory surveillance of patients with PID.However, it is recommended to perform a chest CT scan each 5 years or before any significant therapeutic change. The methods of surveillance need to meet two contradictory imperatives: monitor frequently enough not to diagnose with delay an aggravation of bronchiectasis or interstitial pneumonitis, an infectious complication by a slowly growing pathogen such as a non-tuberculous mycobacterium, or lymphoid proliferation. do not expose these often young patients to significant irradiation by a considerable number of scans during their life. In addition, some patients with PID have increased radiosensitivity without a safe irradiation threshold having been determined. To make thoses requirements effective, the solution is to combine radiological monitoring and absence of irradiation. Therefore, it makes sense to study whether chest scans can be replaced by MRI, non-irradiating imaging. But the question that needs to be answered is whether the information provided by the chest MRI is not inferior to that provided by the scanner. The objective of this study is to assess the ability of MRI performed with ultrashort echo time to analyze the extent and severity of bronchial and pulmonary parenchymal lesions during the follow-up of patients with primary immunodeficiency, comparing them to those of the chest CT scan.

Official Title
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A Prospective Monocentric Study to Assess the Concordance of Lung MRI Compared to Chest CT Scan to Assess the Extent and Severity of Bronchial and Parenchymal Pulmonary Lesions in Adult Patients With Primary Immune Deficiency (PID): IRM vs SCANNER

Conditions
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Primary Immune Deficiency

Intervention / Treatment
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* Other: MRI procedure


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Patients aged 18 or over Adult patients with PID Patients who have performed EFR (Functional Respiratory Tests) within 6 months Patients who have signed a consent form Patients affiliated with a Health Insurance plan. Exclusion Criteria: Pregnant woman Contraindications to MRI: Patients with magnetically activated implanted devices (cardiac pacemakers, insulin pumps, neurostimulators, cochlear implants), Patients with intraocular metal or in the brain (aneurysm clip), Patients with prostheses in the thoracic position and contraindicated for MRI examination Claustrophobic patients. To be deprived of liberty or under guardianship.

Ages Eligible for Study
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Minimum Age: 18 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Design Details

Primary Purpose: Other
Intervention Model: Single Group Assignment
Masking: None (Open Label)


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Experimental: MRI exam<br>Patients will be asked to perform a pulmonary MRI in addition to the usual chest CT can. The chest CT scan will be performed according to the usual protocol of standard practices. No difference is expected for this research protocol. | Other: MRI procedure<br>* Pulmonary MRI based on a 1.5 T machine (Siemens VIDA) or a 3T machine (Siemens SOLA) without the use of contrast product according to a protocol based on a 3D-UTE sequence acquired in free breathing but with a respiratory synchronization system in association with weightings in T1 and T2.<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| To assess the bronchial lesions | Analysis of the presence, the severity (cylindrical, varicose or cystic) and segmental distribution of bronchiectasis, 2. the presence and lobar distribution of hypoperfused pulmonary areas, and 3. the presence and lobar distribution of interstitial abnormality and pulmonary nodular lesions. Those three components will be independently identified by lung scanners and MRIs. | 6 montths | 
| To assess the parenchymal lesions | Analysis of the presence and lobar distribution of hypoperfused pulmonary areas | 6 montths | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| To assess the MRI results versus the scanner results | To assess the reproducibility of MRI performed using a prototype 3D-UTE SPIRALVIBE sequence and CT scan to detect bronchial and pulmonary parenchymal abnormalities at the segmental, lobar or pulmonary scale, by two independent thoracic radiologists. | 6 months | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Primary Immune Deficiency, MRI, Chest CT Scan