Chronic Inflammatory Demyelinating Polyneuropathy

Although there are close to 10 randomized trials showing efficacy for prednisone, intravenous immunoglobulin, or plasmapheresis in chronic inflammatory demyelinating polyneuropathy (CIDP), large differences in cost, side effect profiles, and ease of use create controversy over the therapy that is best. Most clinicians use intravenous immunoglobulin or prednisone as first-line therapy. Unfortunately, the clinical trials performed to date are not easily extrapolated to answer, "which agent is best for clinical practice" because they have used varying doses and duration of therapy, different diagnostic criteria for CIDP, and mixtures of patients who have failed previous therapy versus those with newly diagnosed disease. There are a number of small case series describing the efficacy of immune-modulating agents, such as azathioprine, cyclophosphamide, cyclosporine A, interferon-alpha, and mycophenolate mofetil. These studies generally use these medications as second-line agents for patients who failed corticosteroids or intravenous immunoglobulin. In the authors' practice, this lack of robust knowledge still relegates these to second-line or prednisone-sparing agents.