An update on the pathogenesis and treatment for CIDP

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  • Koike Haruki
    Department of Neurology, Nagoya University Graduate School of Medicine

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  • 慢性炎症性脱髄性多発ニューロパチーの病態と治療法の最新情報

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<p>Chronic inflammatory demyelinating polyneuropathy (CIDP) is a form of chronic neuropathy caused by heterogeneous immununological processes. In addition to the typical form of CIDP, several atypical forms, such as distal acquired demyelinating symmetric (DADS), multifocal acquired demyelinating sensory and motor, focal, pure motor, and pure sensory types, have also been included as subtypes of CIDP. Demyelination caused by macrophages has been reported in CIDP and is considered to play an important role in the pathogenesis of this disease irrespective of subtypes. By contrast, recent studies have suggested the association of IgG4 antibodies directed against paranodal junction proteins, such as anti–neurofascin 155 and anti–contactin 1 antibodies, to subpopulations of typical CIDP and DADS patients. Paranodal dissection resulting from the attachment of IgG4 at paranodal junctions and the absence of macrophage–induced demyelination are the characteristic pathological features in patients with these antibodies. Therefore, two distinct mechanisms that lead to nerve conduction abnormalities exist from a pathological viewpoint in CIDP. Regarding the treatment of CIDP, maintenance therapy using intravenous or subcutaneous administration of immunoglobulin has become available. Advances in the search for autoantibodies and development of new therapeutic options may make it necessary to reconsider the classification of CIDP and establish a long–term therapeutic strategy based on the mechanisms of this disease.</p>

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