[Topics of chronic inflammatory demyelinating polyradiculoneuropathy--diagnosis and treatment situation in Japan].

CIDP is a motor and sensory neuropathy characterized by chronic, step-wised, or relapsing progression. Both cellular and humoral autoimmunity targeting the myelin sheath is assumed as the main mechanism of CIDP pathogenesis. While the AAN diagnostic criteria have been the main method in Japan, the EFNS/PNS criteria recently replaced them because of their good diagnostic sensitivity and clinical superiority. The first-line therapy for CIDP patients in Japan is intravenous immunoglobulin (IVIg), corticosteroids, and phasmapheresis, the same as in other countries. Regarding therapeutics, two major differences between Japan and other countries exist. Firstly, while half-dose IVIg (1 g/kg body weight) every three weeks was established as maintenance therapy as a result of the ICE study in 2008, full-dose IVIg (2 g/kg body weight over five days) once a month is still accepted in Japan for highly recurrent patients. Secondly, Japanese clinicians prefer immune adsorption plasmapheresis (IAPP) instead of plasma exchange (PE) among three types of plasmapheresis (IAPP, PE, and double-filtered plasmapheresis [DFPP]). These differences could be due to the characteristic and independent health insurance system in Japan. Using recent knowledge and diagnostic criteria, clinical trials have been based on these global platforms. Recently, efforts have been made to share these platforms with a worldwide vision.