Abatacept in Juvenile Dermatomyositis

Inclusion criteria

• . Adults with definite or probable JDM and pediatric patients 7 years of age and older with definite or probable JDM.
• . Body weight of at least 25 kg (or at least 55 lbs) and age ≥ 7 years of age.
• . Patients must reside within the United States or Canada.
• . Refractory myositis, as defined by the intolerance to or an inadequate response to corticosteroids plus an adequate regimen of at least one other immunosuppressive agent, including azathioprine, methotrexate, IVIG, mycophenolate mofetil, cyclophosphamide, tacrolimus or cyclosporine A, or a biologic therapy. The definition of intolerance is: side effects that require discontinuation of the medication or an underlying condition that precludes the further use of the medication.
• . At least moderately active disease as documented by:
• . Therapy with prednisone or another glucocorticoid is required, unless there is documented intolerance in the medical record or a medical condition that contraindicates further use of prednisone. The prednisone dose must be stable for at least 4 weeks prior to the screening visit.
• . Background therapy with at least 1 non-corticosteroid immunosuppressive agent is required at a stable dose for at least 6 weeks prior to the screening visit unless there is documentation that the patient is intolerant, which is defined as side effects that require discontinuation of the medication(s) or an underlying condition that precludes the further use of the IS medication.
• . If an immunosuppressive agent was discontinued prior to the screening visit then there must be a: