[The use of intravenous immunglobulin in the treatment of autoimmune neuromuscular diseases].

Intravenous immunglobulin given in autoimmune neuromuscular disorders modulates the immune system by complex actions, including, 1. the modification of the expression and function of Fc receptors, 2. interference with the activation of the complement and the cytokine network, 3. neutralisation of antiidiotypic antibodies, 4. effects on the activation, differentiation and effector functions of the T and B cells. Controlled trials have shown that intravenous immunglobulin is effective as first-line therapy in patients with Guillain-Barré syndrome and multifocal motor neuropathy. In case of steroid resistance or coexisting diabetes mellitus, intravenous immunglobulin can be the first line therapy in chronic inflammatory demyelinating polyneuropathy as well. As an alternative therapy it can be a second-line choice in dermatomyositis, myasthenia gravis, Lambert-Eaton myasthenic syndrome, and stiff person syndrome. While it has a remarkably good safety record for long-term administration the following side effects have been observed: headache, skin rash, thromboembolic events and renal tubular necrosis. In some disorders, the appropriate dose and frequency of infusions that maintain a satisfactory therapeutic response is well defined on the basis of data of evidence-based medicine, whereas in others it still remains to be defined. For the analysis of pharmacoeconomical aspects and the mechanism(s) of response differences in the same disease categories, further studies are necessary.