Assessment of Cystic Fibrosis Lung Involvement With UTE Pulse Sequences (MucoIRM)

Assessment of Lung Involvement in Cystic Fibrosis Patients Using 1.5T MR Imaging With Ultrashort Echo Time (UTE) Pulse Sequences

Non-invasive assessment of lung involvement is a crucial issue for the follow-up of cystic fibrosis patients. Currently, CT is the method of reference to evaluate and quantify the lung morphological changes in cystic fibrosis adults but it remains a radiation-based technique. MRI with ultrashort echo time (UTE) pulse sequences is a promising non-ionizing alternative for lung imaging. The investigators' objective is to evaluate cystic fibrosis lung involvement using CT and MRI-UTE, and to test the agreement between both techniques.

Study Overview

• Status: Completed
• Conditions: Cystic Fibrosis
• Intervention / Treatment: Device: MR measurements of lung morphological changes in cystic fibrosis (Avanto dot, Siemens); Device: CT measurements of lung morphological changes in cystic fibrosis

Detailed Description

Cystic fibrosis is a recessive autosomic fatal disease, affecting about 6000 people in France. Thanks to progress in symptomatic care, median survival is increasing. The lung involvement is the most common and responsible for most deaths. The evaluation of respiratory disease severity is based on pulmonary functional tests (PFT) and imaging. Multi-slice computed tomography (CT) is the method of reference to quantify lung involvement in cystic fibrosis. It detects respiratory lung involvement earlier than PFT, and it reveals lesions associated with the onset of respiratory exacerbations, the mortality increase and the reduction of quality of life. However CT provides ionizing radiation, thus limiting the possibility of long-term follow-up. MRI is a non-ionizing 3D imaging technique; nevertheless, lung MRI is technically challenging with the result that it is currently not used in routine practice. Indeed, both low proton density and susceptibility effects lead to very low signal intensity derived from lung parenchyma. Recently, pulse sequences with ultrashort echo time (UTE) have been implemented by the use of half radio-frequency excitations and radial projection reconstruction. These UTE sequences make it theoretically possible to retrieve more signal from the lung parenchyma. The investigators aim at using 3D T1-weighted UTE pulse sequences on a 1.5T magnet (Avanto dot, Siemens) in cystic fibrosis patients in order to assess lung involvement severity. Thirty three cystic fibrosis adults are expected to take part in the study. All will benefit from PFT, CT and MRI. The investigators' strategy is to establish a semi quantitative score of pulmonary severity (Helbich score) using MRI and CT in subjects, testing for correlations between MRI and CT measurements and assessing the reproducibility of lung lesions quantification using MRI. The investigators' objective is to demonstrate that MRI-UTE pulse sequence at 1.5T is accurate and reproducible in evaluating and quantifying pulmonary involvement in cystic fibrosis.

• Study Type: Interventional
• Enrollment (Actual): 30
• Phase: Not Applicable

Contacts and Locations

Study Locations

• France
  • Pessac, France
    • University Hospital Bordeaux

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 18 years and older (Adult, Older Adult)
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

Description

Inclusion Criteria:

• cystic fibrosis adults
• written informed consent

Exclusion Criteria:

• Subjects without any social security or health insurance
• Pregnancy
• MRI contraindications: Magnetically activated implanted devices (cardiac pacemakers, insulin pumps, neurostimulators, cochlear implants), metal inside the eye or the brain (aneurysm clip, ocular foreign body), cardiac valvular prosthesis (Starr-Edwards pre-6000), subject with claustrophobia.

Study Plan

How is the study designed?

Design Details

• Primary Purpose: Supportive Care
• Allocation: N/A
• Interventional Model: Single Group Assignment
• Masking: None (Open Label)

Number of Arms

1

Arms and Interventions

Participant Group / Arm	Intervention / Treatment
Experimental: Cystic fibrosis adults	Device: MR measurements of lung morphological changes in cystic fibrosis (Avanto dot, Siemens); Device: CT measurements of lung morphological changes in cystic fibrosis

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Agreement between MRI and CT for scoring lung involvement in cystic fibrosis	MRI and CT evaluation of lung lesions quantification in cystic fibrosis will be assessed by 2 blind radiologists. Agreement between both evaluation will be assessed during statistical analyses after study completion.	During MRI and CT, Day one

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Cystic fibrosis lesions scoring extracted from CT and MR images	MRI and CT evaluation of lung lesions quantification in cystic fibrosis will be assessed by 2 blind radiologists. Agreement between both evaluation will be assessed during statistical analyses after study completion.	During MRI and CT, Day one
MRI cystic fibrosis reproductibility scoring over the time	MRI cystic fibrosis scoring will be assessed a second time the same blind radiologist	During MRI, Day one
Correlation between MRI cystic fibrosis scoring and PFT indexes		Day one
Correlation between MRI cystic fibrosis scoring and exacerbation number		12 month after Day one
Correlation between MRI cystic fibrosis scoring and quality of life		Day one and 12 month after Day one
Evolution of MRI cystic fibrosis scoring after one year		12 month after Day one

Collaborators and Investigators

• Sponsor: University Hospital, Bordeaux

Publications and helpful links

General Publications

• Benlala I, Point S, Leung C, Berger P, Woods JC, Raherison C, Laurent F, Macey J, Dournes G. Volumetric quantification of lung MR signal intensities using ultrashort TE as an automated score in cystic fibrosis. Eur Radiol. 2020 Oct;30(10):5479-5488. doi: 10.1007/s00330-020-06910-w. Epub 2020 May 15.

Study record dates

Study Major Dates

• Study Start (Actual): September 30, 2015
• Primary Completion (Actual): September 6, 2016
• Study Completion (Actual): September 6, 2017

Study Registration Dates

• First Submitted: May 13, 2015
• First Submitted That Met QC Criteria: May 19, 2015
• First Posted (Estimate): May 20, 2015

Study Record Updates

• Last Update Posted (Actual): November 27, 2017
• Last Update Submitted That Met QC Criteria: November 23, 2017
• Last Verified: November 1, 2017

More Information

Terms related to this study

• Keywords: MRI; Quality of life; CT; Cystic fibrosis; Exacerbation; Pulmonary function; UTE
• Additional Relevant MeSH Terms: Digestive System Diseases; Pathologic Processes; Respiratory Tract Diseases; Lung Diseases; Infant, Newborn, Diseases; Genetic Diseases, Inborn; Pancreatic Diseases; Fibrosis; Cystic Fibrosis
• Other Study ID Numbers: CHUBX2014/11