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?
This is a prospective, single site, observational study in adults aged 18-80 who have fibrotic interstitial lung disease (fILD), and are scheduled to start antifibrotic therapy as prescribed by their treating respirologist. Therapeutic treatment will be assigned in a non-randomized manner by the treating respirologist. There will be no control intervention or blinding. Each participant will participate in three study visits over a one-year period; Visit 1 - before start of treatment Visit 2 - after 24 weeks of therapy Visit 3 - after 48 weeks of therapy Each visit will include; a blood test - Complete Blood Count (CBC), C-reactive Protein (CRP), Erythrocyte Sedimentation Rate (ESR); Pulmonary Function testing (PFT) that includes spirometry, plethysmography, diffusion capacity, oscillometry; urine pregnancy test (if indicated); St George's Respiratory Questionnaire (SGRQ-I) and King's Brief Interstitial Lung Disease (K-BILD) questionnaire; 129Xenon Magnetic Resonance Imaging (129Xe MRI); Low-dose chest Computed Tomography (CT) /High Resolution Computed Tomography (HRCT) scan; Six-Minute Walk Test (6MWT) with Borg rating of perceived exertion and dyspnea (BORG) questionnaires.
Study Type
OBSERVATIONAL
Enrollment
35
Inhalation of hyperpolarized 129Xe gas followed by breath-hold MRI for ventilation imaging.
Robarts Research Institute; The University of Western Ontario; London Health Sciences Centre
London, Ontario, Canada
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.
Time frame: 3 visits; Visit 1 at baseline, before treatment initiation. Visit 2 at 24 weeks post-treatment initiation. Visit 3 at 48 weeks post treatment initiation. Each visit will include MRI and CT imaging, Blood test, PFT, questionnaires and 6MWT.
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.
Time frame: From baseline to 1 year post-therapy initiation.
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