Therapy of CIDP and related immune-mediated neuropathies

This study assesses the data supporting the current use of immunotherapy for management of neuropathies with a presumed autoimmune basis. Immune or inflammatory mechanisms are implicated in an increasing number of disorders that involve damage to peripheral nerves and sensory ganglia. The most prevalent of these is chronic inflammatory demyelinating polyradiculoneuritis (CIDP). CIDP is clinically distinguishable from a series of other immune- or inflammation-mediated neuropathies. These include the following: multifocal acquired demyelinating sensory and motor (MADSAM) neuropathy associated with conduction block; brachial or lumbar plexitis; multifocal motor neuropathy, a distal demyelinating neuropathy associated with antibodies to the myelin-associated glycoprotein (MAG); and sensory neuronopathy. We reviewed the literature of the National Library of Medicine using the above terms and their variations. The results from short-term, randomized, controlled trials support the efficacy of intravenous immunoglobulin (IVIg), plasma exchange, and corticosteroids in the treatment of CIDP, and the efficacy of IVIg in the treatment of MMN. Only anecdotal experience is available for assessing the treatment of MADSAM neuropathy, lumbar and brachial plexitis, MAG neuropathy, and sensory neuronopathy. Cytotoxic and immunosuppressive drugs are frequently used to treat patients with many of these disorders when they are refractory to standard treatments, but no controlled trials have been done to support their use. This review discusses the complications and criteria for use of these therapies. Selecting the best immunotherapy for an individual patient should be based on the proven efficacy of a given regimen in modulating the pathophysiology underlying the neuropathy, the anticipated duration of disease, and the patient's age, reproductive status, and coexisting metabolic derangement.