What side effects are associated with this type of intervention?

Common treatments for Lupus, particularly those involving monoclonal antibodies and immunosuppressive agents, can have a range of side effects. For example, Rituximab, a monoclonal antibody, has been associated with infusion reactions, serum-sickness-like reactions, and interstitial lung disease. Other immunosuppressive treatments like glucocorticoids can lead to side effects such as increased infection risk, osteoporosis, and hyperglycemia. Emapalumab, being an Anti-Interferon Gamma Monoclonal Antibody, is expected to have similar immunosuppressive side effects, including potential increased susceptibility to infections and infusion-related reactions.

What prior FDA approvals exist?

The FDA has approved several biologic agents for the treatment of Lupus, particularly systemic lupus erythematosus (SLE). One notable approval is Belimumab (Benlysta), a monoclonal antibody that inhibits B-lymphocyte stimulator (BLyS), reducing the activity of B cells. Another approved treatment is Anifrolumab (Saphnelo), which targets the type I interferon receptor, thereby inhibiting the interferon pathway, a key driver in Lupus pathogenesis. These treatments are somewhat related to Emapalumab, as they all involve monoclonal antibodies targeting specific pathways in the immune system to manage Lupus symptoms.

What other types of research exist?

Promising novel alternatives to Emapalumab for Lupus patients include Anifrolumab (an anti-interferon alpha receptor monoclonal antibody) and Belimumab (a monoclonal antibody that inhibits B-lymphocyte stimulator). Both have shown efficacy in clinical trials and are approved for treating systemic lupus erythematosus.

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Monoclonal Antibodies

Emapalumab for MAS in Still's Disease or Lupus (EMERALD Trial)

Phase 3

Waitlist Available

Research Sponsored by Swedish Orphan Biovitrum

Eligibility Criteria Checklist

Specific guidelines that determine who can or cannot participate in a clinical trial

Must have

Confirmed diagnosis of SLE as per SLICC'12 criteria.

Must not have

Confirmed malignancy. Note: subjects with a suspected malignancy should have mononuclear cells typed by flow cytometry and/or tissue biopsy, as applicable, to rule out malignancy.

Treatment with canakinumab, JAK inhibitors, TNF inhibitors and tocilizumab at the time of emapalumab initiation.

Timeline

Screening 3 weeks

Treatment Varies

Follow Up at any time after cr, up to 1 year

Awards & highlights

No Placebo-Only Group

Pivotal Trial

Summary

This trial is testing emapalumab, a medication that helps control an overactive immune system, in children and adults with certain immune conditions who haven't responded to high-dose steroids. The goal is to see if it is safe and effective in reducing excessive inflammation. Emapalumab is a monoclonal antibody that targets interferon-γ and was first approved for treating primary hemophagocytic lymphohistiocytosis (HLH) in patients with refractory, recurrent, or progressive disease.

Who is the study for?

This trial is for children and adults aged 6 months to 80 years with macrophage activation syndrome (MAS) in Still's disease or systemic lupus erythematosus (SLE), who haven't responded well to high-dose steroids. Participants must meet specific criteria including fever, low platelets, high liver enzymes, and others. Pregnant women, those with certain infections or allergies to the study drug, or recent recipients of live vaccines cannot join.

What is being tested?

The trial tests Emapalumab's safety and effectiveness in treating MAS associated with Still's disease or SLE when standard steroid treatments fail. It involves patients receiving Emapalumab under controlled conditions to see if it improves their symptoms compared to previous treatments.

What are the potential side effects?

Potential side effects of Emapalumab may include allergic reactions due to hypersensitivity, increased risk of infections due to immune system suppression, and possibly other unknown risks that are common when testing new medications.

Eligibility Criteria

Inclusion Criteria

You may be eligible if you check “Yes” for the criteria below

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I have been diagnosed with lupus according to the SLICC'12 criteria.

Exclusion Criteria

You may be eligible for the trial if you check “No” for criteria below:

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My cancer diagnosis has been confirmed.

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I am not taking canakinumab, JAK inhibitors, TNF inhibitors, or tocilizumab when starting emapalumab.

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I have been diagnosed with a leishmania infection.

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I have a genetic form of HLH or a family history of it.

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I do not have active infections like TB, histoplasmosis, or salmonella.

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I am currently taking more than 4 mg/kg of anakinra.

Timeline

Screening ~ 3 weeks3 visits

Treatment ~ Varies

Follow Up ~ at any time after cr, up to 1 year

Screening ~ 3 weeks

Treatment ~ Varies

Follow Up ~at any time after cr, up to 1 year

This trial's timeline: 3 weeks for screening, Varies for treatment, and at any time after cr, up to 1 year for reporting.

Treatment Details

Study Objectives

Study objectives can provide a clearer picture of what you can expect from a treatment.

Primary study objectives

Proportion of subjects with complete response (CR) at Week 8 after first administration of emapalumab

Secondary study objectives

GCs tapering to a dose below 50% of prednisolone (PDN) equivalent at the time of emapalumab start or to the same (or lower) dose being administered before the occurrence of MAS whichever occurs first

Gas Chromatography

MAS recurrence at anytime after achievement of CR

+6 more

Other study objectives

Adverse Events (AEs) (serious and non-serious).

Immunogenicity endpoints

Laboratory parameters

+13 more

Side effects data

From 2021 Phase 2 & 3 trial • 58 Patients • NCT02069899

29%

Viral upper respiratory tract infection

14%

Dental caries

14%

Cholelithiasis

14%

Headache

14%

Still's disease

Condition aggravated

Nausea

Epstein-Barr virus infection

Rhinovirus infection

Rash erythematous

Enterovirus infection

Viral infection

Adverse event following immunisation

Arthritis

Osteonecrosis

Dysphonia

Oedema peripheral

Pyrexia

Arthralgia

Oropharyngeal pain

Urticaria

Lymphopenia

Skin laceration

Rash papular

Rectal prolapse

Cestode infection

Blood alkaline phosphatase increased

100%

80%

60%

40%

20%

Study treatment Arm

Enrolled-06 Cohort

Enrolled-CU Cohort

Enrolled-04 Cohort

Awards & Highlights

No Placebo-Only Group

All patients enrolled in this study will receive some form of active treatment.

Pivotal Trial

The final step before approval, pivotal trials feature drugs that have already shown basic safety & efficacy.

Trial Design

1Treatment groups

Experimental Treatment

Group I: Cohort 1 (sJIA and AOSD) and Cohort 2 (SLE)Experimental Treatment1 Intervention

MAS in the context of systemic juvenile idiopathic arthritis and adult onset Still's disease (sJIA and AOSD) or SLE

Treatment

First Studied

Drug Approval Stage

How many patients have taken this drug

Emapalumab

2014

Completed Phase 3

~110

0 / 7Eligibility criteria met

Research Highlights

Information in this section is not a recommendation. We encourage patients to speak with their healthcare team when evaluating any treatment decision.

Mechanism Of Action

Side Effect Profile

Prior Approvals

Other Research

Common treatments for Lupus include hydroxychloroquine, corticosteroids, and immunosuppressive agents like azathioprine and mycophenolate mofetil. Hydroxychloroquine works by modulating the immune system and reducing inflammation. Corticosteroids suppress the immune response and decrease inflammation rapidly. Immunosuppressive agents inhibit the proliferation of immune cells that attack the body's tissues. Emapalumab, an anti-interferon gamma monoclonal antibody, targets and neutralizes interferon gamma, a cytokine involved in the inflammatory response. This is particularly important for Lupus patients as it helps to reduce the overactive immune response, potentially decreasing disease activity and preventing organ damage.

The 2018 pipeline of targeted therapies under clinical development for Systemic Lupus Erythematosus: a systematic review of trials.New Trials in Lupus and where Are we Going.Natural killer T cells in families of patients with systemic lupus erythematosus: their possible role in regulation of IGG production.