Immunoglobulins in the treatment of neuromuscular autoimmune disorders

Immunoglobulins have been widely used for treatment of neuromuscular autoimmune disorders. Therapeutic efficacy has. been shown in controlled studies in Guillain-Barre syndrome (GBS), chronic polyneuritis (CIDP) and dermoromyositis. in addition, controlled studies have been successfully performed in Lambert-Eaton myasthenic syndrome and multifocal motor neuropathy. In myasthenia gravis, plasmapheresis seems to be superior to intravenous immunoglobulin treatment. By immunoglobulin therapy, a delay of disease progression could be achieved in inclusion body myositis. Possible mechanisms of action have been characterized in animal models and cell culture. antiidiotypic effects, suppression of B- and T-cell activation, blockade of Fc-receptors on phagocytic cells and increased catabolism of pathogenic immunoglobulins, neutralisation of superantigen and complement mediated effects, regulation of cytokine production and cytokine neutralisation, and, most recently, neutralization of blocking antibodies in GBS. Only few side effects have been reported. A widespread use of immunoglobulins is limited by high costs and restricted availability Therefore, alternative therapies have to be considered and in any case of a chronic neuromuscular disease, the lowest effective dose has to be titrated on on individual bose with regular clinical follow-up examinations.