Immunopathogenesis and Treatment of Guillain-Barre Syndrome and Chronic Inflammatory Demyelinating Polyneuropathy

Abstract

This chapter discusses the clinical manifestations, pathogenesis, and treatment of Guillain-Barre syndrome (GBS) and chronic inflammatory demyelinating polyneuropathy (CIDP), including recent advances in immunopathogenesis as well as the treatments in pipeline. GBS and CIDP are part of the spectrum of autoimmune-mediated neuropathy. The target antigen remains elusive in CIDP and acute inflammatory demyelinating polyneuropathy, the most common variant of GBS in North America. Autoimmunity against different gangliosides is involved in the pathogenesis of GBS variants, i.e., acute motor axonal neuropathy, Miller Fisher syndrome, and acute motor and sensory axonal neuropathy, as well as their sub-variants. As GBS is a monophasic disease, immunomodulatory treatment, currently consisting of intravenous immunoglobulin infusions or plasma exchange, is required only during the acute phase, unless there are relapses. On the other hand, maintenance treatment with the aforementioned treatment modalities, as well as corticosteroids or other immunosuppressants, is frequently needed in CIDP patients. Further advances in the understanding of immunopathogenesis of these diseases will likely result in development of new treatments involving targeted immunotherapy.