A Study of Efgartigimod in Patients With IgG4-Related Disease

Stanford University

Status and phase

Enrolling

Phase 2

Conditions

IgG4-related Disease

Treatments

Drug: Efgartigimod

Study type

Interventional

Funder types

Other

Identifiers

NCT07025330

79851

Details and patient eligibility

About

The goal of this clinical trial is to learn if efgartigimod can treat IgG4-related disease in adults. The main questions it aims to answer are:

In patients with IgG4-related disease, does treatment with efgartigimod reduce the volume of the:

lacrimal gland(s) and/or
salivary gland(s) and/or
pancreas

Participants will:

Receive efgartigimod once weekly for up to 12 weeks
Visit the clinic every one to six weeks for checkups and tests
Be asked to complete questionnaires to see how they feel on efgartigimod

Enrollment

5 estimated patients

Sex

All

Ages

18 to 90 years old

Volunteers

No Healthy Volunteers

Inclusion and exclusion criteria

Key Inclusion Criteria:

Have a clinical diagnosis of IgG4-related disease that requires treatment in the opinion of the investigator
Meet the 2019 ACR/EULAR Classification Criteria for IgG4-Related Disease
Have a serum IgG4 concentration greater than or equal to 2 times the upper limit of normal at Screening
Have involvement of the lacrimal gland(s), salivary gland(s), and/or pancreas
- If lacrimal and/or salivary glands are involved, it must be symptomatic, including but not limited to discomfort, pain, dryness, headache, or vision changes
- If the pancreas is involved, it must be asymptomatic, diffuse enlargement without signs or symptoms of obstruction or evidence of major organ dysfunction in the opinion of the investigator
Have a prior inadequate response to, or intolerance of, glucocorticoids, or who have experienced recurrent symptoms after previous treatment with glucocorticoids
Are not receiving current treatment with immunosuppressive medications
All women must test negative for pregnancy and agree to use a reliable method of birth control

Key Exclusion Criteria:

Any exclusion criteria listed in the 2019 ACR/EULAR Classification Criteria for IgG4-Related Disease
Prior treatment with an FcRn inhibitor
Have conventional synthetic disease-modifying antirheumatic drug (csDMARD) or immunosuppressive use as follows:
- Treatment with glucocorticoids within 28 days prior to Baseline or planned treatment during the study
- Treatment with csDMARDs including but not limited to hydroxychloroquine, methotrexate, leflunomide, or sulfasalazine within 28 days prior to Baseline or planned treatment during the study
- Treatment with cytotoxic or immunosuppressive drugs including but not limited to cyclophosphamide, mycophenolic acid, azathioprine, cyclosporine, sirolimus, or tacrolimus within 28 days prior to Baseline or planned treatment during the study
- Treatment with a janus kinase (JAK) inhibitor including but not limited to tofacitinib, baricitinib, upadacitinib, or filgotinib within 28 days prior to Baseline or planned treatment during the study
- Treatment with a Bruton's tyrosine kinase (BTK) inhibitor including but not limited to ibrutinib, zanubrutinib, acalabrutinib, pirtobrutinib, or rilzabrutinib within 28 days prior to Baseline or planned treatment during the study
Have biologic disease-modifying antirheumatic drug (bDMARD) use as follows:
- Treatment with etanercept, adalimumab, or anakinra within 28 days before Baseline or planned treatment during the study
- Treatment with infliximab, certolizumab pegol, golimumab, abatacept, or tocilizumab within 56 days before Baseline or planned treatment during the study
- Treatment with a B cell depleting agent including but not limited to rituximab, ocrelizumab, obinutuzumab, ofatumumab, inebilizumab, ianalumab, or obexelimab ≤ 6 months prior to Baseline
- Patients who received B-cell targeted therapy > 6 and ≤ 12 months prior to Baseline must have a B-cell count that is within the laboratory reference range at Screening
- Treatment with a BAFF antagonist including but not limited to belimumab or tabalumab within 6 months before Baseline or planned treatment during the study
- Treatment with an IL-17 antagonist including but not limited to secukinumab, ixekizumab, or brodalumab within 6 months before Baseline or planned treatment during the study
- Prior treatment with other bDMARDs may be allowed at the discretion of the investigator
A history of, or current, inflammatory or autoimmune disease (that could affect the interpretation of safety or efficacy outcomes) other than IgG4-related disease
Evidence of active tuberculosis, HIV, or hepatitis B or C infection
History of cancer except for skin basal or squamous cell carcinoma, cervical dysplasia or carcinoma in situ that has been treated and is considered cured > 1 year prior to Baseline, prostate cancer considered cured for > 5 years with a normal prostate specific antigen, or colon cancer considered cured > 5 years