Emapalumab for the Treatment of Anti-MDA5 Antibody Positive Rapidly Progressive Interstitial Lung Disease
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.
• Progressive interstitial lung disease as determined by at least 2/4 of the following:
‣ worsening respiratory symptoms;
⁃ worsening or new oxygen requirement;
⁃ worsening disease on CT chest;
⁃ worsening Forced Vital Capacity (FVC1) or Diffusing Capacity for Carbon Monoxide (DLCO) on pulmonary function tests
• MDA5 antibodies present
• Elevated ferritin above the upper limit of normal (ULN)
• Participant at least 18 years old