Update on the treatment of chronic inflammatory demyelinating polyradiculoneuropathy

Purpose of review Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a chronic and often disabling neuropathy, which often responds to immune therapies including corticosteroids, plasma exchange, and high-dose intravenous immunoglobulin (IVIg). The reasons for choosing one of these therapies and the factors that may predict response to these therapies remain, however, unclear. It is also unclear whether other immune therapies may be useful in CIDP. We will here review the studies addressing these points in CIDP. Recent findings Recent studies have shown that IVIg are initially more frequently effective than steroids in CIDP even if steroids, when effective, have a more prolonged efficacy. Some clinical and immunological features including the presence of antibodies against proteins at the nodes of Ranvier were associated with specific response to therapy even if the data need to be confirmed in large series of patients. A few anecdotal studies also reported the efficacy in some patients of new immune therapies whose efficacy needs to be confirmed in controlled trials. Summary IVIg are initially more frequently effective than steroids in CIDP, but the latter have a more prolonged efficacy. Some clinical presentations and their association with specific antibodies reactions may help in predicting the response to specific treatment. The possible benefit of new immune therapies await confirmation from randomized studies.