High-dose intravenous immunoglobulin therapy in dysimmune neuropathies

Abstract

Immune-mediated neuropathies, including Guillain-Barre syndrome, Miller Fisher syndrome, chronic inflammatory demyelinating neuropathy, multifocal motor neuropathy, neuropathies associated with monoclonal gammopathy, and paraneoplastic subacute sensory neuronopathy (Table 1), are a group of disorders believed to be caused by an immune response directed against peripheral nerve antigens. The role of the immune system in the pathogenesis of these neuropathies has been first convincingly demonstrated by Latov et al. in 1980 [1] in a patient with neuropathy associated with IgM monoclonal gammopathy, whose IgM were found to react with the myelin-associated glycoprotein (MAG), a minor component of nerve myelin. Since then antibody reactivities against a number of peripheral nerve antigens have been reported in patients with different forms of dysimmune neuropathies, contributing not only to clarify their pathogenesis [2] but also to their identification and diagnosis. Several immune therapies including steroids, plasma exchange (PE) and a number of immunosuppressive agents, have been reported to be effective in these neuropathies. These therapies are however far from being invariably effective and may be associated with a number of side effects which limit their use, leading to the search for new immune therapies. In particular, in the last decade high-dose intravenous immunoglobulin (IVIg) therapy has been shown to be particularly effective in some of these neuropathies, broadening the spectrum of therapeutical options for these diseases (reviewed in [3, 4]). We review the rationale for using IVIg therapy and the results of its application in these neuropathies, in order to give some indications for its use in these diseases.