Idiopathic Pulmonary Fibrosis
Conditions
Brief summary
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. Participants 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 participants will have repeat studies at 3, 6 and 12 months following the initiation of therapy.
Interventions
DRUGHyperpolarized 129 Xenon Gas Comparing Idiopathic Pulmonary Fibrosis (IPF) Treatment
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
Sponsors
Duke University
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
No
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
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Efficiency of Gas Exchange as Measured by the Red Blood Cell (RBC):Barrier Ratio Following Initiation of IPF Therapy | Baseline, 3, 6, and 12 months following initiation of IPF therapy | RBC:barrier ratio will be determined using 129 Xenon MRI. The RBC:barrier ratio is a key metric that quantifies the efficiency of gas exchange, specifically the balance between red blood cell uptake and alveolar-capillary barrier function by showing how well oxygen gets from the air sacs to the blood. A low ratio often indicates impaired gas transfer, reflecting conditions where either RBC function or the barrier's permeability is compromised. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Change in Pulmonary Function Following Initiation of IPF Therapy - Forced Vital Capacity (FVC) | Baseline, 3, 6, and 12 months following initiation of IPF therapy | FVC, or Forced Vital Capacity, is a lung function test that measures the maximum amount of air a person can forcefully exhale from their lungs after taking a deep breath. This test is performed using a spirometer and the results are used by healthcare providers to help diagnose and monitor pulmonary diseases like asthma, emphysema, or restrictive lung conditions. |
| Change in Pulmonary Function Following Initiation of IPF Therapy - Diffusion Capacity for Carbon Monoxide (DLCO) | Baseline, 3, 6, and 12 months following initiation of IPF therapy | The DLCO, or Diffusing Capacity of the Lung for Carbon Monoxide, is a pulmonary function test that measures how well oxygen transfers from the lungs to the blood by measuring how much carbon monoxide diffuses from the lungs into the bloodstream. Performed by inhaling a special gas mixture and holding the breath, the DLCO test assesses the gas exchange function of the lungs. |
Countries
United States
Contacts
PRINCIPAL_INVESTIGATORRobert Tighe, MD
Duke University Health Systems
Participant flow
Participants by arm
| Arm | Count |
|---|---|
| Newly Diagnosed Idiopathic Pulmonary Fibrosis (IPF) 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
Hyperpolarized 129 Xenon Gas Comparing Idiopathic Pulmonary Fibrosis (IPF) Treatment: 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 | 25 |
| Total | 25 |
Baseline characteristics
| Characteristic | Newly Diagnosed Idiopathic Pulmonary Fibrosis (IPF) |
|---|---|
| Age, Continuous | 72.56 years STANDARD_DEVIATION 6.37 |
| Ethnicity (NIH/OMB) Hispanic or Latino | 1 Participants |
| Ethnicity (NIH/OMB) Not Hispanic or Latino | 24 Participants |
| Ethnicity (NIH/OMB) Unknown or Not Reported | 0 Participants |
| Race (NIH/OMB) American Indian or Alaska Native | 0 Participants |
| Race (NIH/OMB) Asian | 1 Participants |
| Race (NIH/OMB) Black or African American | 1 Participants |
| Race (NIH/OMB) More than one race | 0 Participants |
| Race (NIH/OMB) Native Hawaiian or Other Pacific Islander | 0 Participants |
| Race (NIH/OMB) Unknown or Not Reported | 0 Participants |
| Race (NIH/OMB) White | 23 Participants |
| Region of Enrollment United States | 25 Participants |
| Sex: Female, Male Female | 10 Participants |
| Sex: Female, Male Male | 15 Participants |
Adverse events
| Event type | EG000 affected / at risk |
|---|---|
| deaths Total, all-cause mortality | 0 / 34 |
| other Total, other adverse events | 0 / 34 |
| serious Total, serious adverse events | 0 / 34 |
Outcome results
Primary
Efficiency of Gas Exchange as Measured by the Red Blood Cell (RBC):Barrier Ratio Following Initiation of IPF Therapy
RBC:barrier ratio will be determined using 129 Xenon MRI. The RBC:barrier ratio is a key metric that quantifies the efficiency of gas exchange, specifically the balance between red blood cell uptake and alveolar-capillary barrier function by showing how well oxygen gets from the air sacs to the blood. A low ratio often indicates impaired gas transfer, reflecting conditions where either RBC function or the barrier's permeability is compromised.
Time frame: Baseline, 3, 6, and 12 months following initiation of IPF therapy
| Arm | Measure | Group | Value (MEAN) | Dispersion |
|---|---|---|---|---|
| Newly Diagnosed Idiopathic Pulmonary Fibrosis (IPF) | Efficiency of Gas Exchange as Measured by the Red Blood Cell (RBC):Barrier Ratio Following Initiation of IPF Therapy | Baseline | 0.250 RBC:barrier ratio | Standard Deviation 0.062 |
| Newly Diagnosed Idiopathic Pulmonary Fibrosis (IPF) | Efficiency of Gas Exchange as Measured by the Red Blood Cell (RBC):Barrier Ratio Following Initiation of IPF Therapy | 3 months | 0.237 RBC:barrier ratio | Standard Deviation 0.071 |
| Newly Diagnosed Idiopathic Pulmonary Fibrosis (IPF) | Efficiency of Gas Exchange as Measured by the Red Blood Cell (RBC):Barrier Ratio Following Initiation of IPF Therapy | 6 months | 0.246 RBC:barrier ratio | Standard Deviation 0.073 |
| Newly Diagnosed Idiopathic Pulmonary Fibrosis (IPF) | Efficiency of Gas Exchange as Measured by the Red Blood Cell (RBC):Barrier Ratio Following Initiation of IPF Therapy | 12 months | 0.256 RBC:barrier ratio | Standard Deviation 0.063 |
Comparison: Null hypothesis: there is no difference in the Xenon MRI measure of RBC-to-Membrane (RBC:M) at baseline vs. 3 months after initiating anti-fibrotic therapy.p-value: 0.27t-test, 2 sided
Secondary
Change in Pulmonary Function Following Initiation of IPF Therapy - Diffusion Capacity for Carbon Monoxide (DLCO)
The DLCO, or Diffusing Capacity of the Lung for Carbon Monoxide, is a pulmonary function test that measures how well oxygen transfers from the lungs to the blood by measuring how much carbon monoxide diffuses from the lungs into the bloodstream. Performed by inhaling a special gas mixture and holding the breath, the DLCO test assesses the gas exchange function of the lungs.
Time frame: Baseline, 3, 6, and 12 months following initiation of IPF therapy
Population: Participants with data collected at both timepoints used for analysis. Two participants only completed the baseline visit.
| Arm | Measure | Group | Value (MEAN) | Dispersion |
|---|---|---|---|---|
| Newly Diagnosed Idiopathic Pulmonary Fibrosis (IPF) | Change in Pulmonary Function Following Initiation of IPF Therapy - Diffusion Capacity for Carbon Monoxide (DLCO) | Baseline to 3 months | 0.026 mL/min/mmHg | Standard Deviation 1.961 |
| Newly Diagnosed Idiopathic Pulmonary Fibrosis (IPF) | Change in Pulmonary Function Following Initiation of IPF Therapy - Diffusion Capacity for Carbon Monoxide (DLCO) | Baseline to 6 months | -0.029 mL/min/mmHg | Standard Deviation 2.53 |
| Newly Diagnosed Idiopathic Pulmonary Fibrosis (IPF) | Change in Pulmonary Function Following Initiation of IPF Therapy - Diffusion Capacity for Carbon Monoxide (DLCO) | Baseline to 12 months | -0.306 mL/min/mmHg | Standard Deviation 1.846 |
p-value: 0.95Wilcoxon (Mann-Whitney)
p-value: 0.92Wilcoxon (Mann-Whitney)
p-value: 0.56Wilcoxon (Mann-Whitney)
Secondary
Change in Pulmonary Function Following Initiation of IPF Therapy - Forced Vital Capacity (FVC)
FVC, or Forced Vital Capacity, is a lung function test that measures the maximum amount of air a person can forcefully exhale from their lungs after taking a deep breath. This test is performed using a spirometer and the results are used by healthcare providers to help diagnose and monitor pulmonary diseases like asthma, emphysema, or restrictive lung conditions.
Time frame: Baseline, 3, 6, and 12 months following initiation of IPF therapy
Population: Two participants only completed the baseline visit.
| Arm | Measure | Group | Value (MEAN) | Dispersion |
|---|---|---|---|---|
| Newly Diagnosed Idiopathic Pulmonary Fibrosis (IPF) | Change in Pulmonary Function Following Initiation of IPF Therapy - Forced Vital Capacity (FVC) | Baseline to 12 months | 0.190 liters | Standard Deviation 0.256 |
| Newly Diagnosed Idiopathic Pulmonary Fibrosis (IPF) | Change in Pulmonary Function Following Initiation of IPF Therapy - Forced Vital Capacity (FVC) | Baseline to 3 months | 0.073 liters | Standard Deviation 0.244 |
| Newly Diagnosed Idiopathic Pulmonary Fibrosis (IPF) | Change in Pulmonary Function Following Initiation of IPF Therapy - Forced Vital Capacity (FVC) | Baseline to 6 months | 0.063 liters | Standard Deviation 0.229 |
p-value: 0.23Wilcoxon (Mann-Whitney)
p-value: 0.26Wilcoxon (Mann-Whitney)
p-value: 0.68Wilcoxon (Mann-Whitney)