Elotuzumab in Immunoglobulin G4-Related Disease (IgG4-RD)

Exclusion Criteria

• •1. Presence of a condition other than IgG4-RD that (e.g., asthma) is likely to require systemic Glucocorticoids (GC) for disease control during the period of the trial.
• •2. Malignancy within 5 years (except successfully treated in situ cervical cancer, resected squamous cell or basal cell carcinoma of the skin.)
• •3. The following lab values as indicators of hepatic dysfunction:
• •1. Serum aspartate aminotransferase (AST) or alanine aminotransferase (ALT) greater than three times the upper limit of normal (ULN)
• •2. Total bilirubin \> two times the ULN unless caused by Gilbert's disease. Gilbert's disease with total bilirubin \> three times ULN.
• •3. Serum albumin \< 2.5 gm/dL.
• •4. Evidence of another uncontrolled condition which, in the judgment of the investigator, could interfere with participation in the trial according to the protocol.
• •5. Active infection requiring hospitalization or treatment with systemic antimicrobial agents within the 30 days prior to treatment allocation/randomization.
• •6. Prior use of rituximab or other B cell depleting agents within 9 months of enrollment unless B cells have been demonstrated to have repopulated.
• •7. Use of any investigational agent or biologic and non-biologic DMARDs within 5 half-lives of the agent (or 6 months if the half-life is unknown) prior to enrollment.
• •8. Any of the following laboratory tests at the Screening Visit:
• •1. White blood cell count (WBC) \< 3.0 x 103/µL.
• •2. Absolute neutrophil count (ANC) \< 1.5 x 103/µL.
• •3. Hemoglobin \< 10 g/dL.
• •4. Platelet count \< 75 x 109/L.
• •5. Estimated glomerular filtration rate (eGFR) ≤ 45 ml/minute/1.73 m2.
• •9. The use of supplemental oxygen at baseline.
• •10. At or within 90 days of screening: Positive Interferon-Gamma Release Assay (IGRA). Indeterminate IGRAs must be repeated (with same or other IGRA per local policy) and shown to be negative. Alternatively, if the assay remains indeterminant, a participant must have a negative PPD. Finally, if the participant has had the Bacille Calmette-Guerin (BCG) vaccine or has some other condition complicating the interpretation of TB testing, consultation with infectious disease specialist must be obtained before receipt of the first investigational infusion.
• •a. Participants diagnosed with latent TB are eligible but must have received appropriate prophylaxis for 30 days before their first investigational infusion.
• •11. Medical history or serologic evidence at Screening of chronic infections including:
• •1. Human immunodeficiency virus infection.
• •2. Hepatitis B as indicated by surface antigen or hepatitis B core antibody positivity
• •3. Hepatitis C as indicated by anti-hepatitis C antibody positivity; if a participant is Hepatitis C antibody positive, they will be eligible to participate in the study if he/she is negative for viral load at Screening.
• •12. Live vaccines within 8 weeks of initiating study therapy.
• •13. Participant is pregnant or breastfeeding, or planning a pregnancy while enrolled in the study.
• •14. Substance use disorder, including the recurrent use of alcohol and/or drugs within the past year associated with clinically significant impairment associated with failure to meet major responsibilities at work, school, or home.
• •15. IgG4-RD that is dominated primarily by advanced fibrotic lesions. Specifically, participants whose disease manifestations consist only of
• •1. retroperitoneal fibrosis,
• •2. fibrosing mediatinitis,
• •3. sclerosing mesenteritis, or
• •16. Evidence a SARS-CoV-2 (COVID-19) infection started within the 30 days prior to treatment allocation/randomization. Participants diagnosed with SARS-CoV-2 (COVID-19) infection more than 30 days prior to treatment allocation/randomization must have symptoms resolved and be deemed fit to participate in the trial.