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NCT Number: NCT07486869

Emapalumab MDA5 Rapidly Progressive Interstitial Lung Disease (RP-ILD) Study

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.

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Key information

Conditions

Dermatomyositis Amyopathic dermatomyositis Connective Tissue Diseases Dermatomyositis Sine Myositis Dermatomyositis With Myopathy Dermatomyositis With Organ Involvement Dermatomyositis With Respiratory Involvement Interstitial Lung Disease Interstitial Lung Disease Due to Connective Tissue Disease (Disorder) Lung Diseases Lung Diseases, Interstitial Muscular Diseases Musculoskeletal Diseases Myositis Nervous System Diseases Neuromuscular Diseases Polymyositis Respiratory Tract Diseases Skin Diseases Skin and Connective Tissue Diseases

Age range

18 year and older

Sex eligibility

All sexes

Study type

Interventional

Phase

Phase 2

Primary location

University of Miami Hospital and Clinics

Miami, Florida, 33136, United States

Location contact

Kelly Corbitt, D.O.

CONTACT

[email protected]

305-243-7545

Who can participate

Healthy volunteers accepted: No

Only the study team can determine whether someone qualifies for participation.

Inclusion criteria

  • 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

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

Treatment and study plan

Emapalumab

Drug

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.

Primary outcomes

  1. Change in oxygen requirement

    Time frame: Baseline, 4 weeks, 12 weeks

    Supplemental oxygen requirement assessed as the oxygen flow rate (reported in liters per minute [L/min]) while the participant is at rest and, when feasible, while the participant is ambulating during the 6-Minute Walk Test (6MWT). Values are compared with baseline to assess change over time.

  2. Forced Vital Capacity (FVC)

    Time frame: Baseline, 12 weeks

    Pulmonary function assessed using forced vital capacity (FVC) measured by standard pulmonary function testing (spirometry). The outcome is expressed as percent change from baseline (%) in FVC.

  3. Diffusing capacity of the lungs for carbon monoxide (DLCO)

    Time frame: Baseline, 12 weeks

    Pulmonary gas exchange capacity assessed using diffusing capacity of the lungs for carbon monoxide (DLCO) measured by standard pulmonary function testing. The outcome is expressed as percent change from baseline (%) in DLCO

  4. Change in chest computed tomography (CT) consolidations

    Time frame: Baseline, 4 weeks, 12 weeks

    Pulmonary consolidations assessed on chest computed tomography (CT) scans. CT images are reviewed by a radiologist and pulmonologist, and percentage of lung affected by consolidations will be documented. Percentage change of affected lung will be evaluated.

Secondary outcomes

  1. Change in Ferritin level

    Time frame: Baseline, 4 weeks, 12 weeks

    Serum ferritin concentration measured using standard clinical laboratory testing and reported in nanograms per milliliter (ng/mL). Values obtained at baseline and during post-baseline assessments are used to assess change from baseline in ferritin level.

  2. Change in MDA5 antibody level

    Time frame: Baseline, 12 weeks

    Serum anti-melanoma differentiation-associated protein 5 (anti-MDA5) antibody level measured as a quantitative serum laboratory value using a validated clinical assay. Values obtained at baseline and during post-baseline assessments are used to assess change from baseline in serum anti-MDA5 antibody level.

  3. New infections during treatment

    Time frame: Baseline, 12 weeks

    Number of participants who experience at least one new infection during the study period, as identified through clinical assessment.

Study contacts

Contact information is provided by the study sponsor or research team.

Kelly Corbitt, D.O.

CONTACT

[email protected]

305-243-7545

Sponsors and collaborators

Lead sponsor

University of Miami

Other

Collaborators

  • Swedish Orphan Biovitrum

Registry information

Official study title

Emapalumab for the Treatment of Anti-MDA5 Antibody Positive Rapidly Progressive Interstitial Lung Disease

Important dates

Study start
2026
Primary completion
2027
Study completion
2027
First posted
Mar 23, 2026
Registry last updated
Jun 18, 2026

OpenTrials presents study information sourced from ClinicalTrials.gov. The official registry record should be consulted for the latest information.

View the official ClinicalTrials.gov record (opens in a new tab)

This listing is for discovery and informational purposes only. It is not medical advice, does not guarantee that a study is recruiting, and does not determine eligibility. Contact the study team and a qualified healthcare professional when considering participation.

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