Belimumab in Idiopathic Inflammatory Myositis

Exclusion Criteria

• •Subjects meeting any of the following criteria will be excluded from the study:
• •1. Have severe muscle damage as defined by a Muscle Damage Index (MDI) \> 5.0 cm using a visual analogue scale (VAS) 10.0 cm in length or evidence of severe muscle atrophy on muscle MRI.
• •2. History of malignant neoplasm within the last 5 years, except for adequately treated cancers of the skin (basal or squamous cell).
• •3. Have a history of a primary immunodeficiency
• •4. Have a significant IgG deficiency (IgG level \< 400 mg/dl) Have an IgA deficiency (IgA level \< 10 mg/dL)
• •5. Discontinuation IS agent \< 3 months prior to Screening. Including: azathioprine, methotrexate, mycophenolate mofetil, leflunomide, tacrolimus, cyclosporine, or intravenous gamma globulin \[IVIG\]
• •6. Have received Rituximab within 365 days prior to Screening.
• •7. Have received cyclophosphamide within 180 days prior to Screening
• •8. Have received treatment with:
• •1. Initiated IVIG 3 months prior to Screening
• •2. Pulse steroids 2 months prior to Screening
• •9. Have received treatment with Belimumab at any time prior to Screening
• •10. Have received a biologic investigational agent within 365 days prior to Screening.
• •11. Have received a non-biologic investigational agent within 30 days or 5 half-lives of the agent (whichever is longer) prior to Screening.
• •12. Have a history of an anaphylactic reaction to parenteral administration of contrast agents, human or murine proteins or monoclonal antibodies.
• •13. Infection history:
• •1. Currently on any suppressive therapy for a chronic infection (such as tuberculosis - including latent tuberculosis, pneumocystis, cytomegalovirus, herpes simplex virus, herpes zoster and atypical mycobacteria).
• •NOTE: Testing for latent TB is a standard of care for patients on immunosuppressive therapy and results will be obtained from their medical record. If no TB history is found for these patients, a Quantiferon Gold or PPD test will be performed prior to Day 0 as part of standard of care.
• •2. Hospitalization for treatment of infection within 60 days of Day 0.
• •3. Use of parenteral (IV or IM) antibiotics (antibacterials, antivirals, anti-fungals, or anti parasitic agents) within 60 days of Day 0.
• •14. Have a historically positive HIV test or test positive at screening for HIV.
• •15. Have a history of autoimmune hepatitis
• •16. Hepatitis status will be obtained from patients' medical records. If unavailable, testing will be done as part of standard of care. Patients are excluded if there is evidence of infection with:
• •a. Hepatitis B: i. Positive hepatitis B surface antigen (HBsAg) or positive hepatitis B core antibody (HBcAb) or b. Hepatitis C: i. Positive hepatitis C antibody with confirmatory hepatitis C viral load by PCR.
• •17. Clinically significant elevation of GGT (\>1.5xULN), bilirubin (\>1.25xULN, direct 35%), or INR (\>1.2, excluding patients on anti-coagulant therapies) or other clinically significant abnormal laboratory value in the opinion of the investigator.
• •18. Have current drug or alcohol abuse or dependence, or a history of drug or alcohol abuse or dependence within 364 days prior to Day 0.
• •19. Have evidence of serious suicide risk including any history of suicidal behaviour in the last 6 months and/or any suicidal ideation in the last 2 months or who in the investigator's judgment, pose a significant suicide risk.
• •20. Have any concurrent significant medical or psychiatric illness that the investigator considers would make the candidate unsuitable for the study.