Chronic Inflammatory Demyelinating Polyneuropathy

Treatment of chronic inflammatory demyelinating polyneuropathy (CIDP) requires long-term immunomodulatory therapy, which has potential side effects. Before such therapy is instituted, a firm diagnosis of CIDP must be established. Prednisone, plasma exchange (PE), and intravenous immunoglobulin (IVIG) are all proven first-line therapies for CIDP that are of similar efficacy. The choice of treatment should be individualized based on costs, availability, and potential adverse effects. Except in the elderly and in those with complicating medical illnesses, IVIG is well tolerated and easy to administer; hence, it should be the initial therapy for most patients with CIDP. Because of its prohibitive costs and limited availability, however, it is not ideal for long-term administration. In the elderly and in those with complicating medical illnesses (eg, diabetes, obesity, or hypertension), PE may be used as the first-line therapy. Because the effects of PE are transient and because it is expensive, requires vascular access, and can only be performed in specialized centers, long-term therapy with PE alone is problematic. Prednisone is inexpensive, easily available, and of proven efficacy. It is the preferred treatment in young, otherwise healthy persons either as a first-line therapy or in association with IVIG or PE. Patients who require repeated treatment with IVIG or PE and cannot tolerate prednisone or those who require high-dose prednisone should be administered azathioprine, cyclosporin A, or cyclophosphamide, usually in combination with one of the first-line therapies.