muscle inflammation myositis
Conditions
Interventions
Administration of 2 gram/kg IVIg at baseline after 4 and 8 weeks (intervention
arm), or placebo (Saline 0.9%) infusions at baseline and after 4 and 8 weeks
(control arm). All patients will be treate
Sponsors
Academisch Medisch Centrum
Eligibility
Age
18 Years to 99 Years
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 • Signed informed consent
Exclusion criteria
Exclusion criteria: • Disease duration > 12 months • Immunosuppressive medication within the last 12 months (azathioprine, methotrexate plasmapheresis, IVIg, biologicals). We will allow prednisone dosed as follows: - Daily dose 20 mg or lower, used for two weeks or less - Daily dose higher than 20 mg, used for 1 week or less - No evident clinical response • 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) • 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 |
|---|---|
| The Total Improvement Score (TIS) of the myositis response criteria after week 12 compared to baseline t=0, before treatment. Total Improvement Score is based on 6 validated core set measures which each determine disease activity as defined by the International Myositis Assessment and Clinical Studies (IMACS) group. | — |
Secondary
| Measure | Time frame |
|---|---|
| 1. Time to response (response defined as Total Improvement Score >40 points) 2. Total improvement score (IMACS) 3. Each of the cores set measures out of which IMACS TIS is composed. 4. Patient-Reported Outcomes. Three PROMs of the Patient Reported Outcomes Measurement Information System (PROMIS) will be used. These PROMs relate to different aspects of quality of life in patients with myositis: fatigue, pain interference and physical function. 5. Health related quality of life assessed with EuroQol Group Health Questionnaire (EQ5D). 6. Physical activity measured by accelerometry. 7. Fatigue via CIS Fatigue questionaire 8. Mean daily prednisone dosage 9. Muscle hyperintensities and fatty infiltration on total body MRI (T1 and T2/STIR) 10. IgG blood levels 11. Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI) in the subgroup of patients with dermatomyositis | — |
Countries
Netherlands
Outcome results
None listed