Treatment of patients with chronic inflammatory demyelinating polyneuropathy.

Patients with a chronic inflammatory demyelinating polyneuropathy (CIDP) have progressive weakness of at least 2 months. In randomized trials, efficacy has been shown for prednisone, plasma exchange (PE) and intravenous immunoglobulin (IVIg). A recent trial showed equal efficacy for PE and IVIg. Since IVIg has no serious side effects and is relatively easy to administer, it may be the first treatment choice. IVIg treatment is shown to be effective in the majority, but not in all patients with CIDP. The proportion of patients that improves after IVIg seems to be roughly up to 70-90%. Patients with signs and symptoms of an active disease and clear involvement of both arms and legs appear to have the highest chance to improve after IVIg treatment, but additional or prospective studies are needed to verify these criteria. Most patients with initial improvement need intermittent IVIg treatment to maintain their improved clinical condition. Prognostic factors are analyzed now that may predict not only the initial chance to respond after IVIg treatment, but also the necessity for long-term intermittent treatment to prevent relapse. New studies should not only compare the efficacy and safety of IVIg with other treatments during the initial treatment period, but should also look for the cost-benefit ratio during long-term treatment regimens.