Emapalumab for fast-worsening lung disease linked to anti-MDA5 dermatomyositis

AI summary

From the trial's eligibility criteria. Machine-read from the registry text on this page, so it can be wrong. The registry text below and the trial team decide who can take part.

The trial is looking for adults with lung scarring disease (interstitial lung disease) that is getting worse quickly and who have MDA5 antibodies. It tests the drug Emapalumab.

Age

18 years and older.

Sex

All sexes can join.

Disease stage

Interstitial lung disease that is getting worse, shown by at least 2 of 4 signs: worse breathing symptoms, new or higher need for oxygen, worse disease on a chest CT scan, or worse results on lung function tests (FVC1 or DLCO).

Biomarker or test result

MDA5 antibodies must be present. Ferritin in the blood must be above the upper limit of normal.

Main reasons for not taking part

• Active bacterial, mycobacterial or fungal infection that is not treated
• Active herpes zoster (shingles) infection
• Currently needing ECMO, a machine that adds oxygen to the blood outside the body
• Pregnant women
• Prisoners

The registry criteria, word for word

What the trial wants to find out

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.

The group in the trial

• EmapalumabTreatment being tested

  Participants will receive emapalumab administered intravenously at a dose of 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 an additional 2 weeks. Total treatment duration is 12 weeks.