Idiopathic Pulmonary Fibrosis, Hypersensitivity Pneumonitis
Conditions
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.
Interventions
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.
Sponsors
Duke University
Children's Hospital Medical Center, Cincinnati
Boehringer Ingelheim
University of Kansas Medical Center
Study design
Allocation
NA
Intervention model
SINGLE_GROUP
Primary purpose
DIAGNOSTIC
Masking
NONE
Eligibility
Sex/Gender
ALL
Age
18 Years to No maximum
Healthy volunteers
Yes
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
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| RBC:Barrier Ratio | RBC:Barrier will be assessed at Baseline and at 6 months. | 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. |
Outcome results
None listed