Immunoglobulin treatment in refractory myasthenia gravis

Failure to induce and maintain remission in severe exacerbations of myasthe nia gravis (MG), despite optimal care, is a common problem. We evaluated th e efficacy and safety of high-dose intravenous immunoglobulin (IVIg) therap y in an open-label study of 10 patients with severe generalized myasthenia and an acute deterioration unresponsive to conventional therapy including h igh-dose corticosteroids, cyclosporine, and azathioprine. Intravenous Ig at a loading dose of 400 mg/kg was administered daily for 5 consecutive days, with maintenance IVIg treatment at a dose of 400 mg/kg, once every 6 weeks . Significant improvement occurred in all patients, beginning at 6 +/- 2 da ys of treatment as measured by the Osserman scale, fatigue variables, muscl e strength, and respiratory function tests. No side effects were observed d uring induction of remission. Further IVIg treatments were highly efficacio us in maintaining the remission. The severity of the disease decreased by 2 .5 +/- 0.8 grades of the Osserman scale over a period of 1 year (P <0.001), in parallel with reduction of immunosuppressive therapy as well as a decre ase in acetylcholine receptor antibody titers (P < 0.01). intravenous Ig th erapy seems to be highly potent for inducing rapid improvement in refractor y myasthenia during acute deterioration as well as for maintaining remissio n. (C) 2000 John Wiley & Sons, Inc.