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Immunoglobulin therapy in inflammatory myopathies
F L Mastaglia1, B A Phillips, P J Zilko
1Australian Neuromuscular Research Institute, University Department of Medicine, Queen Elizabeth II Medical Centre, Perth WA.
Journal of Neurology, Neurosurgery, and Psychiatry
|July 17, 1998
Summary
Intravenous immunoglobulin (IVIg) shows promise as an add-on therapy for drug-resistant inflammatory myopathies like polymyositis and dermatomyositis, leading to disease remission and normalized creatine kinase levels in most patients.
Area of Science:
- Neurology
- Immunology
- Rheumatology
Background:
- Inflammatory myopathies, including polymyositis and dermatomyositis, often present treatment challenges.
- Some patients experience disease progression or relapse despite conventional therapies.
Purpose of the Study:
- To evaluate the efficacy of add-on intravenous immunoglobulin (IVIg) therapy in patients with refractory inflammatory myopathy.
- To assess treatment response using objective measures like myometry and serum creatine kinase levels.
Main Methods:
- A prospective, open-label trial involving 16 patients with inflammatory myopathy.
- A three-month run-in period preceded IVIg administration.
- Response assessment included isometric myometry, functional scales, MRC grading, and serum creatine kinase.
Main Results:
- Five of seven patients with polymyositis or dermatomyositis achieved partial or complete remission with normalized creatine kinase.
- All four patients with overlap syndromes responded favorably to IVIg therapy.
- No functional improvement was observed in inclusion body myositis patients, though some myometry improvements occurred in one case.
Conclusions:
- IVIg is a potential therapeutic option for drug-resistant polymyositis and dermatomyositis.
- Further controlled trials are necessary to confirm efficacy and determine optimal dosing and administration protocols for IVIg in inflammatory myopathies.