Fibrotic Interstitial Lung Disease
Conditions
Keywords
Hyperpolarized 129Xe MRI, MRI, Pulmonary Function Testing, fILD, Fibrotic Interstitial Lung Disease, Antifibrotic Therapy, Therapeutic Response
Brief summary
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?
Detailed description
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.
Interventions
Inhalation of hyperpolarized 129Xe gas followed by breath-hold MRI for ventilation imaging.
Sponsors
Dr. Grace Parraga
Study design
Observational model
COHORT
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
18 Years to 80 Years
Healthy volunteers
No
Inclusion criteria
* 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. * Women of childbearing potential must ensure that they are using an effective form of birth control for at least 2 months prior to each imaging visit. * Women of childbearing potential must agree to use a highly effective form of birth control from enrollment, throughout the study duration, with negative urine pregnancy test result at Visits 1, 2, and 3.
Exclusion criteria
* 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. * Participant is unable to perform MRI and CT breath-hold maneuvers.
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Measure 129Xe imaging ventilation defects qualitatively and quantitatively in participants diagnosed with fibrotic interstitial lung disease. | 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. | 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. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| 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. | From baseline to 1 year post-therapy initiation. | 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. |
Countries
Canada
Contacts
CONTACTGrace E Parraga, PhD
CONTACTAndraena K Tilgner, BHSc., RRT
PRINCIPAL_INVESTIGATORGrace E Parraga, PHD
Robarts Research Institute, The University of Western Ontario
Outcome results
None listed