[Immunotherapy of chronic inflammatory demyelinating polyneuropathy]

Chronic inflammatory demyelinating polyneuropathy (CIDP) is an acquired immune-mediated disease of the peripheral nervous system with an estimated prevalence of 1-2/100,000. The clinical presentation is heterogeneous, but the most common form causes symmetrical progressive or relapsing weakness affecting proximal and distal muscles. CIDP is among the most treatable peripheral nerve disorders and corticosteroids, plasmapheresis and intravenous immunoglobulin have been shown to be effective in short-term prospective, randomized controlled trials. Data however indicate that approximately one-third of patients do not respond to these treatment modalities, nor do they provide equivalent evidence for a durable clinical response. There is a lack of good quality controlled trials of any other immunosuppressive agent, but cyclophosphamide and cyclosporin may be of value in patients with poor response to first-line modalities. Alternatively, the use of combination therapy may increase the efficacy in unresponsive patients. This review highlights the current status of CIDP treatment trials and discusses the significance of any therapeutic option in terms of efficacy, tolerability and cost-effects.