Autoimmune Autonomic Disorders

Steven Vernino, MD, PhD, FAAS, FAAN Autonomic Disorders p. 44-57 February 2020, Vol.26, No.1 doi: 10.1212/CON.0000000000000812
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PURPOSE OF REVIEW Autonomic disorders sometimes occur in the context of systemic autoimmune disease or as a direct consequence of autoimmunity against the nervous system. This article provides an overview of autonomic disorders with potential autoimmune etiology.

RECENT FINDINGS Recent evidence highlights a close association between the autonomic nervous system and inflammation. The autonomic nervous system regulates immune function, and autonomic manifestations may occur in a number of systemic autoimmune diseases. In a few instances, autoimmunity directly influences autonomic function. Autoimmune autonomic ganglionopathy is the prototypic antibody-mediated autonomic disorder. Over time, a better understanding of the clinical spectrum of autoimmune autonomic ganglionopathy, the significance of ganglionic nicotinic acetylcholine receptor antibodies, other immune-mediated autonomic neuropathies, and autonomic manifestations of other systemic or neurologic autoimmune disorders has emerged.

SUMMARY Autoimmune autonomic disorders may be challenging, but correct identification of these conditions is important. In some cases, potential exists for effective immunomodulatory treatment.

Address correspondence to Dr Steven Vernino, University of Texas Southwestern Department of Neurology and Neurotherapeutics, 5323 Harry Hines Blvd, Dallas, TX 75390, [email protected].

RELATIONSHIP DISCLOSURE: Dr Vernino serves on the board of directors of the American Autonomic Society; the scientific advisory boards of Argenx, Dysautonomia International, and The Multiple System Atrophy Coalition; and the editorial boards of Autonomic Neuroscience: Basic & Clinical and Clinical Autonomic Research. Dr Vernino receives research/grant support from Dysautonomia International; Genentech, Inc; Grifols, SA; the Rex Griswold Foundation; and Theravance and licensing fees to support his laboratory from Quest Diagnostics Inc.

UNLABELED USE OF PRODUCTS/INVESTIGATIONAL USE DISCLOSURE: Dr Vernino discusses the unlabeled/investigational use of immunomodulatory treatments for autoimmune disorders.

© 2020 American Academy of Neurology.