Emapalumab for the Treatment of Anti-MDA5 Antibody Positive Rapidly Progressive Interstitial Lung Disease
University of Miami
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
- Age range
- 18+ years
- Sex
- All
- Healthy volunteers
- No
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 re…
Interventions
- DrugEmapalumab
Emapalumab administered intravenously according to the following dosing regimen: 6 mg/kg on Day 1, followed by 3 mg/kg every 3 days for 2 weeks, and then 3 mg/kg twice weekly for 2 weeks.
Location
- University of Miami Hospital and ClinicsMiami, Florida