Autoimmunity in the peripheral nervous system.

Abstract

Autoimmune disorders of the peripheral nervous system (PNS) comprise a heterogeneous group of diseases that result from an aberrant immune response. Most of these disorders present severe morbidity and, in some cases, mortality. Even those conditions that are self-limited may display severe disability and necessitate hospitalization. Although their etiology remains elusive, there is increasing knowledge of the pathophysiological mechanisms causing tissue dysfunction and structural damage. The discovery of several mediators that constitute the molecular mechanisms of cell-cell and cell-extracellular-matrix interactions has revealed insight into various aspects of the neuroimmune interaction. Classic animal models associated with new genetic approaches have further increased our comprehension of the molecular pathways that regulate inflammatory disorders of the nervous system. The aim of this review is to describe various types and functions of the principal molecular components of the neuroimmune interaction and their importance in the principal autoimmune disorders of the PNS. We also provide an extensive description of clinical and pathological features of autoimmune disorders of the PNS, along with diagnostic and therapeutic implications.