Chronic Inflammatory Demyelinating Polyneuropathies: Current Treatment Strategies

Chronic inflammatory demyelinating polyradiculopathy (CIDP), considered an immune-mediated disease, is likely under-recognized and under-treated due to its heterogeneous presentation and the limitations of clinical, serologic, and electrophysiologic diagnostic criteria. Despite these limitations, early diagnosis and treatment is important in preventing irreversible axonal loss and improving functional recovery. Primary treatment modalities include intravenous immunoglobulin and plasmapheresis, for which there is randomized, double-blind, placebo-controlled evidence. In addition, despite less definitive published evidence of efficacy, corticosteroids are considered standard therapies because of their long history of use. Studies have failed to demonstrate a difference in efficacy among these three treatments; consequently, the choice is usually based on availability and side-effect profile. A number of chemotherapeutic and immunosuppressive agents have also shown to be effective in treating CIDP but significant evidence is lacking; therefore, these agents are primarily used in conjunction with other modalities. Regardless of the treatment choice, long-term therapy is required to maintain a response and prevent relapse.