2026 年 43 巻 1 号 p. 19-23
Guillain–Barré syndrome (GBS), chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), and multifocal motor neuropathy (MMN) are representative immune–mediated neuropathies in which both humoral and cellular immunity contribute to peripheral nerve injury. Although intravenous immunoglobulin (IVIg) and plasma exchange (PE) remain the established standard therapies for GBS, novel disease–specific treatments such as complement inhibitors are now being developed. In CIDP, the identification of antibodies against paranodal proteins―neurofascin 155, contactin 1, and Caspr1―has led to the recognition of autoimmune nodopathy (AN) as a distinct entity with unique clinical and therapeutic profiles. The approval of the FcRn inhibitor efgartigimod for CIDP marks a paradigm shift toward targeted molecular therapy. In MMN, complement–mediated pathology triggered by anti–GM1 IgM antibodies underlies selective motor conduction block, and IVIg remains the only established treatment, though complement inhibitors are under clinical investigation.
Recent progress in understanding disease mechanisms has clarified the roles of antibody–mediated complement activation, FcRn–dependent IgG recycling, and B–cell immunity in these disorders. For CIDP and AN, B–cell–depleting therapy with rituximab has shown benefit, particularly in IgG4 antibody–positive subtypes, while phase 3 trials of FcRn and complement inhibitors are expanding treatment options. Moreover, advanced immunomodulatory strategies such as autologous hematopoietic stem cell transplantation and CD19–directed CAR–T cell therapy are being explored for refractory cases.
Collectively, these advances highlight a transition in the management of inflammatory neuropathies―from nonspecific immunosuppression to precise, pathophysiology–based interventions. Future research should focus on biomarker–driven stratification and individualized treatment approaches to achieve durable remission and functional recovery.
