Chronic immune-mediated neuropathies, including chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), neuropathies associated with monoclonal gammopathy, and multifocal motor neuropathy (MMN), are a group of disorders deemed to be caused by an immune response against peripheral nerve antigens. Several immune therapies have been reported to be variably effective in these neuropathies including steroids, plasma exchange, and high-dose intravenous (IVIg) or subcutaneous (SCIg) immunoglobulins. These therapies are however far from being invariably effective and may be associated with a number of side effects leading to the use of immunosuppressive agents whose efficacy has not been so far confirmed in randomized trials. More recently, new biological agents, such as rituximab, have proved to be effective in patients with neuropathy associated with IgM monoclonal gammopathy and are currently tested in CIDP.
Keywords: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP); Immune-mediated neuropathies; Intravenous or subcutaneous immunoglobulins; Multifocal motor neuropathy (MMN); Neuropathies; Paraproteinemic; Rituximab.
© 2021. Fondazione Società Italiana di Neurologia.