This is a proof of concept study to determine if Emapalumab appears effective for the treatment of anti-MDA5 antibody positive rapidly progressive interstitial lung disease (MDA5 RP-ILD). Emapalumab is a medication that is currently used for a severe problem with the immune system, called macrophage activation syndrome, and this disease shares some similar features with MDA5 RP-ILD.
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Change in oxygen requirement
Timeframe: Baseline, 4 weeks, 12 weeks
Forced Vital Capacity (FVC)
Timeframe: Baseline, 12 weeks
Diffusing capacity of the lungs for carbon monoxide (DLCO)
Timeframe: Baseline, 12 weeks
Change in chest computed tomography (CT) consolidations
Timeframe: Baseline, 4 weeks, 12 weeks