Novel Immunological and Therapeutic Insights in Guillain-Barré Syndrome and CIDP

Abstract

Inflammatory neuropathies are a heterogeneous group of rare diseases of the peripheral nervous system that include acute and chronic diseases, such as Guillain-Barré syndrome (GBS) and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). The etiology and pathophysiological mechanisms of inflammatory neuropathies are only partly known, but are considered autoimmune disorders in which an aberrant immune response, including cellular and humoral components, is directed towards components of the peripheral nerve causing demyelination and axonal damage. Therapy of these disorders includes broad-spectrum immunomodulatory and immunosuppressive treatments, such as intravenous immunoglobulin, corticosteroids, or plasma exchange. However, a significant proportion of patients do not respond to any of these therapies, and treatment selection is not optimized according to disease pathophysiology. Therefore, research on disease pathophysiology aiming to reveal clinically and functionally relevant disease mechanisms and the development of new treatment approaches are needed to optimize disease outcomes in CIDP and GBS. This topical review describes immunological progress that may help guide therapeutic strategies in the future in these two disorders. Supplementary Information The online version contains supplementary material available at 10.1007/s13311-021-01117-3.