Therapeutic progress and future prospects of chronic inflammatory demyelinating polyradiculoneuropathy

Abstract

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a rare and heterogeneous but treatable immune-mediated neuropathy. The pathogenesis of CIDP is complex interplay of diverse immune mechanisms involving cellular and humoral pathways. The European Academy of Neurology/Peripheral Nerve Society guidelines were reissued in 2021, and the classification and diagnostic criteria were changed. Treatments include immunoglobulin, steroid, and plasmapheresis are effective, including remission induction and maintenance therapy. Maintenance treatments are often required for years, and treatment regimens require careful and regular adjustments to avoid undertreatment or overtreatment. In this review, the new guidelines, treatment recommendations based on guidelines and expert opinion, and future treatments including anti CD20 monoclonal antibody, FcRn blocker, and Cs1 inhibitor are discussed.