Add-on Intravenous Immunoglobulins in Early Myositis (TIMEISMUSCLE)
Inflammatory Myopathy, Idiopathic, Dermatomyositis, Antisynthetase Syndrome
About this trial
This is an interventional treatment trial for Inflammatory Myopathy, Idiopathic focused on measuring Myositis, Inflammatory myopathies, Intravenous immunoglobulins, Early symptomatic
Eligibility Criteria
Inclusion Criteria: Adult patients (โฅ 18 years) with IIM, according to diagnostic criteria: Dermatomyositis Polymyositis Anti-synthetase syndrome Immune mediated necrotizing myopathy Overlap myositis Disease duration < 12 months Minimal disability defined as at least 10% loss on Manual Muscle Testing (MMT) and abnormal scores on two other Core Set Measures (CSMs) of the international Myositis Assessment and Clinical Studies (IMACS) group (see 'Primary and secondary outcomes'). Patients are eligible for inclusion if they are treatment-naive, or if there is no clinical evident response (as carefully judged by the treating physician at a screening visit) to prior treatment with: High dosed glucocorticoids, such as dexamethasone (e.g. 40 mg per day up to 4 days) or intravenous methylprednisolone (e.g. 1000 mg daily for three days), within 1 week prior to screening visit. Daily dosed prednisone 1 mg/kg, or equivalent, used for up to 2 weeks prior to screening visit. Treatment with low-dosed prednisone (max 20 mg daily) up to three months prior to screening visit. Treatment with biologicals or other immunosuppressive or immunomodulatory treatment when meeting all of the following criteria: Stable dose for the last 6 months The biological or other immunosuppressive or immunomodulatory treatment has been approved for a non-muscular condition (e.g. hematological condition, eczema) and is not known for its use in idiopathic inflammatory myopathy The biological or other immunosuppressive or immunomodulatory treatment is not known to induce inflammatory myopathy Signed informed consent Exclusion Criteria: A potentially eligible patient who meets any of the following criteria will be excluded from participation in this study: Severe muscle weakness (i.e. bedridden, severe dysphagia requiring a feeding tube, or respiratory muscle weakness (forced vital capacity below 50% of predicted in upright position)) necessitating more intensive treatment than standard glucocorticoids from the start. Related to IVIg: History of thrombotic episodes within 10 years prior to enrolment Known allergic reactions or other severe reactions to any blood-derived product Known Immunoglobulin A (IgA) deficiency and IgA serum antibodies Pregnancy or trying to conceive Use of loop diuretics Use of nephrotoxic medication Conditions that are likely to interfere with: Compliance (legally incompetent and/or incapacitated patients are excluded), or, Evaluation of efficacy (e.g. due to severe pre-existing disability as a result of any other disease than myositis or due to language barrier) Immunosuppressive medication or immunomodulatory treatment within the last 3 months (e.g. azathioprine, methotrexate, mycophenolate mofetil, tacrolimus, cyclophosphamide, cyclosporine, IVIg, biologicals, Janus kinase inhibitors, plasmapheresis).
Sites / Locations
- Department of Neurology, Amsterdam UMC, location AMCRecruiting
Arms of the Study
Arm 1
Arm 2
Experimental
Placebo Comparator
Add-on IVIg
Placebo
Patients in the intervention arm will be treated with Nanogamยฎ in a dosage of 2 g/kg over 2-5 days, with a maximum of 80 g/day and a total of 180 gram at baseline and after 4 and 8 weeks. Nanogamยฎ contains 100 mg/mL normal human immunoglobulin with a purity of at least 95% IgG and a maximum of 12 microgram/mL IgA, in a solution of water for injections and glucose. The first 30 grams are administered in hospital.
Patients in the control arm will be treated with placebo infusions, containing sodium chloride 0.9%, at baseline and after 4 and 8 weeks. The first 30 grams are administered at the neurology ward, after 4 and 8 weeks infusions will be administered at home