NCT07486869PHASE2Recruiting
Emapalumab MDA5 Rapidly Progressive Interstitial Lung Disease (RP-ILD) Study
University of Miami
Start Date
7/23/2026
Completion Date
7/1/2027
Summary
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 Criteria
Age Range: 18 years to No maximum
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
Interventions
DRUG
Emapalumab
Conditions
DermatomyositisDermatomyositis Sine MyositisDermatomyositis With MyopathyDermatomyositis With Respiratory InvolvementDermatomyositis With Organ InvolvementInterstitial Lung DiseaseInterstitial Lung Disease Due to Connective Tissue Disease (Disorder)
Locations
University of Miami Hospital and Clinics
Miami, Florida 33136
United States