Intravenousimmunoglobulin (IVIg) for the Treatment of Inflammatory Myopathies

Launched by NATIONAL INSTITUTE OF NEUROLOGICAL DISORDERS AND STROKE (NINDS) · Nov 3, 1999

Keywords

ClinConnect Summary

The inflammatory myopathies are a group of acquired muscle diseases characterized by subacute onset of progressive proximal muscle weakness, elevated serum muscle enzymes and endomysial inflammation. They comprise 3 clinically distinct subsets: polymyositis (PM), dermatomyositis (DM) and Inclusion Body Myositis (IBM). Because immune-mediated mechanisms are primarily responsible for the clinical manifestations of these conditions, the treatment of choice is with corticosteroids or immunotherapy drugs. Although most of the patients initially respond to these drugs, a number of them become res...

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Eligibility criteria

• INCLUSION CRITERIA:
• • Selected patients should have PM, IBM or DM.
• • Specifically they should have a) proximal muscle weakness; b) no evidence of clinical, histological or family history of another neuromuscular illness; c) elevation of muscle enzymes during the course of the disease; d) typical skin rash in case of DM; and e) diagnostic muscle biopsy.
• Suitable candidates for IVIg should be patients with active, bonefide disease who:
• • 1. have been treated with steroids but had: a) no response or incomplete response (as defined by continued muscle weakness) to high-dose therapy or b) a good response to steroids but inability to taper the dose without a flare of disease activity or c) unacceptable steroid side effects such as gastrointestinal hemorrhages, osteonecrosis, hyperglycemia, extreme weight gain etc., and
• • 2. have been treated with one immunosuppressive drug (such as azathioprine, Methotrexate, Cyclophosphamide, Cyclosporine) but without benefit or with unacceptable side effects.
• EXCLUSION CRITERIA:
• • Pregnant or nursing women (confirmed by a screening pregnancy test).
• • Critically ill patients such as those requiring intravenous pressors for maintenance of cardiac output due to severe cardiomyopathy, patients with respiratory insufficiency and patients with severe muscle weakness requiring help for basic self care.
• • Children below age 18.
• • Patients with severe renal or hepatic disease, severe COPD or coronary artery disease or other systemic medical problems often seen when PM or DM is associated with severe cases of lupus, rheumatoid arthritis or scleroderma.
• • Patients with known allergic reaction to IVIg.
• • Serum IgA less than 11mg/dl.