Overview

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.

Eligibility

Inclusion Criteria:

• Progressive interstitial lung disease as determined by at least 2/4 of the following:
  1. worsening respiratory symptoms;
  2. worsening or new oxygen requirement;
  3. worsening disease on CT chest;
  4. 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

Exclusion Criteria:

• Active, untreated bacterial, mycobacterial or fungal infection
• Active herpes zoster infection
• Currently requiring extracorporeal membrane oxygenation (ECMO)
• Participant refusal to participate in the study
• Pregnant women
• Prisoners