Diagnosis and treatment of chronic inflammatory demyelinating polyneuropathy

Chronic inflammatory demyelinating polyneuropathy (CIDP) is an immune-mediated acquired polyneuropathy that may lead to disability. CIDP is characterized by an autoimmune attack against peripheral nervous system myelin, by cellular and humoral mechanisms. Early diagnosis and treatment may yield better functional recovery, probably by minimizing secondary axonal loss from a primary demyelinating insult. Intravenous immunoglobulin and plasmapheresis are considered standard-of-care therapy in CIDP, based on randomized, double-blinded, placebo-controlled evidence. Corticosteroids, despite less robust evidence, are also considered standard therapy for CIDP. Other nonstandard therapies may work in refractory patients. These include azathioprine, cyclophosphamide, cyclosporine A, etanercept, interferon-alpha 2a, mycophenolate mofetil, and tacrolimus. Emerging therapies include interferon-beta 1a, rituximab, and high-dose cyclophosphamide without stem-cell rescue. Because most patients will require prolonged therapy, long-term side effects are important considerations.