慢性炎症性脱髄性多発神経炎(chronic inflammatory demyelinating polyneuropathy : CIDP)の病態と治療(<特集>免疫性神経疾患とアフェレシス,新しい展望)

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  • Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) : Pathogenesis and Therapy

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Chronic inflammatory demyelinating polyneuropathy (CIDP) is chronic or relapsing demyelinating neuropathy, which cause weakness and/or sensory disturbance. The pathogenesis of CIDP is assumed to be immune-mediated, and immunomodulating therapy is effective. Only half of patients can reach a remission state, while the rest of the patients are treatment resistant. The efficacy of each therapy differs from patient to patient : thus it is not easy to make a simple guideline for therapy for CIDP. Corticosteroid therapy, plasmapheresis and high-dose intravenous immunoglobulin therapy (IVIg) are the choice for initial therapy. Almost the same percentage (about 30%) of patients on each therapy can be treated well with single one ; thus it is better to try another therapy if the first is not efficacious enough. In plasmapheresis, double-filtration plasmapheresis or immunoadsorption are used in Japan and seems to have the same efficacy compare with plasma exchange. Quicker response is the advantage of plasma exchange, but relapse often occurs, so there is a usually need to use corticosteroid, IVIg or immuno-suppressive agents.

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