The purpose of this study is to test MRI methods for evaluating patients with Scleroderma-associated interstitial lung disease.
1. Quantify the sensitivity and specificity of UTE MRI in screening for scleroderma-associated interstitial lung disease. ILD is relatively common in SSc, but current clinical standards require screening using HRCT. UTE MRI can provide images of pulmonary structure with contrast and resolution approaching that of CT. We hypothesize that UTE MRI will have high sensitivity and specificity (\>80%) to the presence of ILD as determined by HRCT. Aim 2. Quantify treatment response in patients with SSc-ILD using hyperpolarized 129Xe MRI. The optimal treatment for SSc-ILD is not known, and it is challenging to assess the efficacy of therapy. Hyperpolarized 129Xe MRI is highly sensitive to the pathophysiology associated with ILD, which suggests that it may be more sensitive to treatment response than conventional methods. We hypothesize that hyperpolarized 129Xe MRI biomarkers will be sensitive to lung function improvement or decline earlier than standard clinical measures (6MWD, FVC, DLCO, Dyspnea score).
Study Type
OBSERVATIONAL
Enrollment
25
University of Kansas Medical Center
Kansas City, Kansas, United States
Presence of ILD on CT and MRI
An experienced radiologist will review MRI and CT images to assess whether ILD is present.
Time frame: Baseline
Change in RBC/Barrier Ratio
The change in hyperpolarized 129Xe RBC/Barrier ratio from baseline to follow-up imaging
Time frame: 6 months
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