The goal of this observational study is to better understand how treatment affects the lungs of adults (ages 18-80) with fibrotic interstitial lung diseases. The main questions it aims to answer are:
* Can advanced imaging detect the effect of currently approved therapies on people with fibrotic interstitial lung diseases? * What is the relationship between the response to therapy and the imaging response to therapy?
Patient understands study procedures and is willing to participate in the study as indicated by the patient's signature.
Provision of written, informed consent prior to any study specific procedures.
Males and females >/= 18 years of age
Patient has a current diagnosis of fILD by a respirologist according to the 2022 ATS/ERS guidelines and is eligible for antifibrotic therapy.
Patient has an implanted mechanically, electrically, or magnetically activated device or any metal in their body which cannot be removed, including but not limited to pacemakers, neurostimulators, biostimulators, implanted insulin pumps, aneurysm clips, bioprosthesis, artificial limb, metallic fragment or foreign body, shunt, surgical staples (including clips or metallic sutures and/or ear implants)
In the investigator's opinion, subject suffers from any physical, psychological or other conditions that might prevent performance of the MRI or CT, such as severe claustrophobia.
Participants who are pregnant, breastfeeding or have a positive pregnancy test at initial screening visit.
Participant is unable to perform spirometry or plethysmography maneuvers.
Participants will be adults 18-80 years of age with physician-diagnosed fibrotic interstitial lung disease consistent with 2022 ATS/ERS guidelines and are eligible for antifibrotic therapy. Participants will be referred from respirologists at LHSC London outpatient respirology clinics.
Cohort
Prospective
Hyperpolarized 129Xe MRI
Other interventionInhalation of hyperpolarized 129Xe gas followed by breath-hold MRI for ventilation imaging.
Measure 129Xe imaging ventilation defects qualitatively and quantitatively in participants diagnosed with fibrotic interstitial lung disease.
The extent and timing of clinical and imaging-based physiologic changes following treatment initiation for fibrotic interstitial lung disease will be assessed using conventional assessments and advanced pulmonary imaging (MRI and CT). Measurements will include 129Xe compartment-specific physiologic abnormalities and ventilation defect percent (VDP), Pulmonary Function Test (PFT) including Forced Vital Capacity (FVC), Total Lung Capacity (TLC) and Diffusing Capacity of the Lungs for Carbon Monoxide (DLCO), Six-Minute Walk Test (6MWT), symptom scores from St George's Respiratory Questionnaire (SGRQ-I) and King's Brief Interstitial Lung Disease (KBILD) questionnaires, CT parenchyma/airway/vessel morphology.
Determine the relationships between clinical and imaging response to therapy and generate predictive models for their achievement at 1-year using baseline markers and their changes at 24 weeks.
Observe changes in 129Xe compartment-specific physiologic abnormalities, changes in ventilation defect percent (VDP), Changes in CT (for progression in fibrotic disease), PFT, 6MWT, SGRQ-I, KBILD.
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Dr. Grace Parraga
Lead sponsor
Western University, Canada
Sponsor institution
This trial is not recruiting at the moment. You can still explore other options:
