The current place of high-dose immunoglobulins in the treatment of neuromuscular disorders

Abstract

High-dose immunoglobulins for intravenous administration (IVIg) have originally been developed for substitution therapy in hypogammaglobulinemia. Over the last decade they are increasingly used in the treatment of immune-mediated diseases. In this review the results in immune-mediated neuromuscular diseases are summarized. Positive effects are demonstrated in open studies in dermato- and polymyositis, myasthenia gravis, and inflammatory neuropathies. Properly conducted randomized clinical trials demonstrating the effect of IVIg are available in dermatomyositis, Guillain-Barré syndrome, and chronic inflammatory demyelinating polyneuropathy, and smaller ones in multifocal motor neuropathy. In myasthenia gravis a trial is at present underway and only interim results are available. The results of a trial in the Lambert-Eaton myasthenic syndrome are in the process of publication. The therapeutic approach in individual patients is discussed, but often appears to be difficult. Considering chronic treatment with IVIg, proper long-term studies including cost-benefit studies are needed. Future developments aim for combination therapies, since IVIg and immune suppressants like prednisone are suggested to have a synergistic effect.