Primary Immune Deficiency
Conditions
Keywords
Primary Immune Deficiency, MRI, Chest CT Scan
Brief summary
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.
Interventions
OTHERMRI procedure
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.
Sponsors
Siemens Healthcare QT
Hopital Foch
Study design
Allocation
NA
Intervention model
SINGLE_GROUP
Primary purpose
OTHER
Masking
NONE
Eligibility
Sex/Gender
ALL
Age
18 Years to No maximum
Healthy volunteers
No
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.
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| To assess the bronchial lesions | 6 montths | 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. |
| To assess the parenchymal lesions | 6 montths | Analysis of the presence and lobar distribution of hypoperfused pulmonary areas |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| To assess the MRI results versus the scanner results | 6 months | 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. |
Countries
France
Contacts
Primary ContactElisabeth Hulier-Ammar,PHD
Backup ContactVirginie Guitard
Outcome results
None listed