Ublituximab in Autoantibody Positive Immune Mediated Necrotizing Myopathy
Trial Parameters
Brief Summary
This is a multi-center, Phase 2 trial of ublituximab as first-line combination therapy in early, active autoantibody positive immune-mediated necrotizing myopathy. The primary objective is to estimate the effect of ublituximab as first add-on combination therapy at 24 weeks compared to placebo in treating early, active autoantibody positive immune-mediated necrotizing myopathy using the validated 2016 American College of Rheumatology/ European League Against Rheumatism (ACR/EULAR) Myositis Response Criteria, as measured by the Total Improvement Score (TIS)
Eligibility Criteria
Inclusion Criteria: 1. Participant must be able to understand and provide informed consent. 2. Age 18 years or older at disease onset. 3. Definite or probable IIM per the 2017 EULAR/ACR classification criteria. 4. Diagnosis of IMNM, meeting the 2016 ENMC classification criteria and having either of the following antibodies: 1. Anti-SRP 2. Anti-HMGCR 5. Early disease as defined as onset of objective muscle weakness assessed by a physician and/or CK elevation attributed to IMNM within 1 year of the time of informed consent. 6. Muscle weakness as assessed by an MMT-8 score \< 142 of 150 and CK \> 1.5x ULN along with abnormality in at least 1 of the following 4 CSMs at screening: 1. PhGA ≥ 2 cm 2. PtGA ≥ 2 cm 3. Extramuscular global assessment ≥ 2 cm 4. HAQ-DI ≥ 0.25 7. Treatment with only one of the following background immunosuppressant medications for IMNM for at least 12 weeks prior to randomization and the same dose for at least 4 weeks prior to randomization: 1. Methotrexate up to 25