Interstitial Lung Disease, Pulmonary Hypertension, Systemic Sclerosis
Conditions
Keywords
Interstitial Lung Disease, Pulmonary Hypertension, Magnetic Resonance Imaging, Systemic Sclerosis
Brief summary
This study aims to evaluate a noninvasive magnetic resonance imaging technique using hyperpolarized xenon gas to measure lung function in adults with interstitial lung disease, pulmonary hypertension, and systemic sclerosis. Researchers will assess whether MRI measurements correlate with clinical measures of lung and vascular function.
Interventions
DRUGHyperpolarized 129 Xenon Gas
Hyperpolarized xenon gas is inhaled during MRI to evaluate lung ventilation and gas exchange.
Sponsors
Bastiaan Driehuys
National Heart, Lung, and Blood Institute (NHLBI)
Study design
Allocation
NA
Intervention model
SINGLE_GROUP
Primary purpose
BASIC_SCIENCE
Masking
NONE
Eligibility
Sex/Gender
ALL
Age
18 Years to No maximum
Healthy volunteers
Yes
Inclusion criteria
* Age ≥18 years at enrollment. * Participant is willing and able to provide written informed consent. * Able (in the Investigator's opinion) and willing to comply with all study requirements. * Participant has or is suspected to have one of the following pulmonary conditions: * Healthy volunteers: aged 18-30 years; no known cardiopulmonary disease; has not smoked tobacco products in the previous 5 years; smoking history, if any, is less than or equal to 5 pack-years; no history of using other inhaled products more than 1 time per week for \> 1 year OR * ILD-PH: ≥18 years; ILD diagnosis; precapillary PH by RHC (mPAP \>20 mmHg, PVR \>2 WU, PCWP ≤15 mmHg) within the specified window OR * SSc: systemic sclerosis diagnosis with or without PH
Exclusion criteria
* Any condition which, in the opinion of the Investigator, would make it unsafe or unsuitable for the participants to undergo MRI (for example: presence of an implanted or external MRI unsafe device that cannot be removed, body weight exceeding table limitations, inability to lie flat, or severe claustrophobia). * Inability to hold breath for 10-15 seconds for required imaging or for completion of DLCO measurements. * Active pulmonary infection or lung cancer. * Recent respiratory infection or pulmonary disease exacerbation within 4 weeks of screening. * Participants who are known to be pregnant or lactating. * Concurrent enrollment and participation in a study which, in the opinion of the Investigator, would interfere with the study compliance, or outcome assessments or would prohibit the collection of the required data points.
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Diagnostic Accuracy for Detection of Pulmonary Hypertension (sensitivity) | Single study visit, Day 1 | Sensitivity of pre-specified 129Xe MRI biomarkers for identifying pre-capillary pulmonary hypertension in participants with systemic sclerosis, using right-heart catheterization as the reference standard. |
| Diagnostic Accuracy for Detection of Pulmonary Hypertension (specificity) | Single study visit, Day 1 | Specificity of pre-specified 129Xe MRI biomarkers for identifying pre-capillary pulmonary hypertension in participants with systemic sclerosis, using right-heart catheterization as the reference standard. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| RBC Oscillation Imaging Amplitude Defect Percentage | Single study visit, Day 1 | Percentage of lung volume exhibiting abnormal regional RBC oscillation amplitude as measured by hyperpolarized 129Xe MRI. |
| Whole-Lung RBC Chemical Shift | Single study visit, Day 1 | Mean frequency difference between the center of the RBC resonance and the alveolar gas resonance. |
Contacts
PRINCIPAL_INVESTIGATORJoseph Mammarappallil
Duke University
Outcome results
None listed