Idiopathic inflammatory myopathy, with the exception of inclusion body myositis.
Conditions
Interventions
Three times 2g/kg IVIg or placebo, at 4-week-intervals, directly following a diagnosis of IIM
Sponsors
Prinses Beatrix spierfonds, Sanquin Plasma Products
Eligibility
Inclusion criteria
Inclusion criteria: • Adult patients (18-80 years) with idiopathic inflammatory myopathy (IIM), according to diagnostic criteria: - Dermatomyositis - Non-specific/overlap myositis including antisynthetase syndrome; formerly known as polymyositis - Immune mediated necrotizing myopathy • 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’). • Signed informed consent
Exclusion criteria
Exclusion criteria: A potentially eligible patient who meets any of the following criteria will be excluded from participation in this study: • Disease duration > 12 months • Immunosuppressive medication or immunomodulatory treatment within the last 3 months (e.g. azathioprine, methotrexate, mycophenolate mofetil, tacrolimus, cyclophosphamide, ciclosporine, IVIg, biologicals, Janus kinase inhibitors, plasmapheresis). • Severe muscle weakness (i.e. bedridden, severe dysphagia, or respiratory muscle weakness) necessitating more intensive treatment than standard glucocorticoids. Exceptions to abovementioned exclusion criteria: Prior use of steroids will be carefully judged by the treating physician. Patients are eligible for inclusion if there is no clinical evident response to prior treatment with: • High-dosed steroids, such as dexamethasone or intravenous methylprednisolone (e.g. 1000mg daily for three days) within 1 week prior to inclusion. • Daily dosed prednisone 1mg/kg, or equivalent, used for up to 2 weeks prior to screening visit. • Treatment with low dose prednisone up to three months before screening. • Use of biologicals or other immunosuppressive or immunomodulatory treatment when meeting the following criteria: - Stable dose for the past 6 months - The biological has been approved for a non-muscular condition (e.g. hematological condition, eczema) and is not known for its use in idiopathic inflammatory myopathy - No history of biological-induced inflammatory myopathy • 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 IgA deficiency and IgA serum antibodies - Pregnancy (wish) - Use of loop diuretics - Use of nephrotoxic medication • Conditions that are likely to interfere with: - Compliance (legal 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)
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Change in Total Improvement Score (week 12 compared to baseline) | — |
Secondary
| Measure | Time frame |
|---|---|
| Time to improvement (TIS>40 points), mean prednison dosage, health-related quality of life, fatigue, pain, physical activity, IgG blood levels, muscle MRI, cutaneous dermatomyositis disease area and severity, 'samengestelde vragenlijst zorggebruik en productiviteitsverlies' | — |
Contacts
Public ContactRenske Kamperman
Amsterdam UMC, locatie AMC
Outcome results
None listed