Genentech Xenon MRI Idiopathic Pulmonary Fibrosis

Study Overview
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Brief Summary
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The purpose of this study is being done to determine whether magnetic resonance imaging (MRI) using inhaled hyper-polarized 129 Xenon gas can help visualize impaired lung function to detect changes over time in Idiopathic Pulmonary Fibrosis (IPF) patients receiving approved IPF treatments. Subjects will undergo an approximately hour long comprehensive MRI protocol, including administration of multiple doses of hyper-polarized 129 Xenon. The subjects will have this initial study prior to initiation of IPF therapies. Then the subjects will have repeat studies at 3, 6 and 12 months following the initiation of therapy. Additional studies including pulmonary function studies, serum for bio markers, 6 minute walk distance and a high-resolution computed tomography (HRCT) scan (only at the 6 month visit) will be performed to determine how 129 Xenon MRI performs relative to standard of care evaluations for IPF. The MRI uses a magnet and radio waves to make diagnostic medical images of the body. There have been no ill effects reported from exposure to the magnetism or radio waves used in this test. Risks of the xenon gas are slight numbness in legs, nausea, a feeling of well-being, and mild tingling in fingertips. You will have pulmonary function testing for the study, you may experience breathlessness or dizziness during or immediately following these tests.

Official Title
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Using Xenon MRI to Evaluate the Efficacy of Therapies for Idiopathic Pulmonary Fibrosis

Conditions
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Idiopathic Pulmonary Fibrosis

Intervention / Treatment
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* Drug: Hyperpolarized 129 Xenon Gas Comparing Idiopathic Pulmonary Fibrosis (IPF) Treatment


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Outpatients of either gender, age > 18. Willing and able to give informed consent and adhere to visit/protocol schedules. (Consent must be given before any study procedures are performed) Clinical diagnosis of IPF by confirmed by multidisciplinary diagnosis and naïve to treatment with an approved IPF therapy (either nintedanib or pirfenidone) Exclusion Criteria: Subject is less than 18 years old Subjects who have been previously on either pirfenidone or nintedanib MRI is contraindicated based on responses to MRI screening questionnaire Subject is pregnant or lactating Resting oxygen saturation on room air <90% on supplemental oxygen Respiratory illness of a bacterial or viral etiology within 30 days of MRI Subject with ventricular cardiac arrhythmia in the past 30 days. Subject has history of cardiac arrest within the last year Subject does not fit into 129 Xenon vest coil used for MRI Subject deemed unlikely to be able to comply with instructions during imaging Recent exacerbation (within 30 days) defined by the need for antibiotics and/or systemic steroids Medical or psychological conditions which, in the opinion of the investigator, might create undue risk to the subject or interfere with the subject's ability to comply with the protocol requirements

Ages Eligible for Study
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Minimum Age: 18 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Design Details

Primary Purpose: Diagnostic
Intervention Model: Single Group Assignment
Masking: None (Open Label)


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Experimental: Newly Diagnosed Idiopathic Pulmonary Fibrosis (IPF)<br>Whether magnetic resonance imaging (MRI) using inhaled hyper-polarized 129 Xenon gas can help visualize impaired lung function to detect changes over time in Idiopathic Pulmonary Fibrosis (IPF) patients receiving approved IPF treatments | Drug: Hyperpolarized 129 Xenon Gas Comparing Idiopathic Pulmonary Fibrosis (IPF) Treatment<br>* Whether magnetic resonance imaging (MRI) using inhaled hyper-polarized 129 Xenon gas can help visualize impaired lung function to detect changes over time in Idiopathic Pulmonary Fibrosis (IPF) patients receiving approved IPF treatments<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Change in Red Blood Cell (RBC):Barrier Ratio Following Initiation of IPF Therapy | RBC:barrier ratio will be determined using 129 Xenon MRI. This ratio will be assessed in each individual prior to initiation of IPF therapy. Following initiation of therapy additional 129 Xenon MRIs will be performed at 3, 6 and 12 months. The outcome measure will be assessment of rate of RBC:barrier decline following initiation of therapy across the imaging studies from the initial 129 Xenon MRI performed prior to initiation of therapy. | The RBC:barrier measure will be assessed at time of study enrollment and then at 3, 6 and 12 months following initiation of IPF therapy. | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Change in Pulmonary Function Following Initiation of IPF therapy | Lung function will be assessed in all subjects. This will include forced vital capacity (FVC) and diffusion capacity for carbon monoxide (DLCO). The outcome measure will be for FVC decline >10% or DLCO decline >20% over the 12 months. These measures have been identified as surrogate measures of IPF outcomes in prior clinical studies. | Pulmonary function testing will occur prior to initiation of therapy and then at 3, 6 and 12 months following initiation of therapy. |