129Xe Gas Exchange Imaging in IPF and cHP: A Reliability Study

Study Overview
=================
Brief Summary
-----------------
This research is a study to test the reliability of Hyperpolarized Xenon MRI (HXe MRI) as a biomarker in interstitial lung disease. The study is a non-randomized study to evaluate the test-retest performance of HXe MRI in Idiopathic Pulmonary Fibrosis (IPF) and chronic Hypersensitivity Pneumonitis (cHP) as a non-invasive biomarker of disease severity and prognosis. The study will include approximately 15 subjects with IPF, 15 subjects with cHP and 10 sex and age-matched normal controls performed across 3 sites.

Official Title
-----------------
129Xe Gas Exchange Imaging in IPF and cHP: A Reliability Study

Conditions
-----------------
Idiopathic Pulmonary Fibrosis, Hypersensitivity Pneumonitis

Intervention / Treatment
-----------------
* Drug: Hyperpolarized Xe129


Participation Criteria
=================
Eligibility Criteria
-----------------
Inclusion Criteria: Adults > 18 years of age Diagnosis of progressive chronic HP, IPF as determined by MDD discussion (historical or during screening period) OR normal control (no history of known lung disease, no abnormal parenchymal or airway findings on CT examination and no values outside of the normal for FVC, FEV1, TLC, and DLCO.) FVC % Predicted >45% DLCO % Predicted >30% Progressive lung disease as defined by one of the following criteria within 24 months of screening visit Relative decline in the FVC ≥ 10% of the predicted value Relative decline in the FVC of ≥ 5% - < 10% of the predicted value and worsening of respiratory symptoms Relative decline in the FVC of ≥ 5% - < 10% of the predicted value and an increased extent of fibrosis on prior clinical high-resolution CT Worsening of respiratory symptoms and an increased extent of fibrosis on high-resolution CT. Exclusion Criteria: Stable FVC over 2-year period as determined by study physician Unstable cardiac condition within 6 months of screening as determined by study physician

Ages Eligible for Study
-----------------
Minimum Age: 18 Years

Sexes Eligible for Study
-----------------
All

Accepts Healthy Volunteers
-----------------
Accepts Healthy Volunteers

Study Plan
=================
How is the study designed?
-----------------

Design Details

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


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Experimental: Subjects with Progressive Interstitial Lung Disease<br>Assess the intra-visit reliability and sensitivity to progression of 129Xe MR imaging measurements using a standardized imaging protocol in subjects with IPF and cHP. | Drug: Hyperpolarized Xe129<br>* Whether magnetic resonance imaging (MRI) using inhaled hyper-polarized 129 Xenon gas can help visualize impaired lung function to assess patients with cHP and IPF.<br>* Other names: HP 129Xe;|

What is the study measuring?
-----------------
Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| RBC:Barrier Ratio | RBC:Barrier ratio will be determined using 129 Xenon MRI in 2 successive scans on two separate days 6 months apart. Same-day Repeated measures will be used to assess reliability of RBC:Barrier. Data acquired at 6 months will be correlated with FVC. | RBC:Barrier will be assessed at Baseline and at 6 months. |