Autoimmune autonomic neuropathies and ganglionopathies: epidemiology, pathophysiology, and therapeutic advances

Research output: Contribution to journalReview article

3 Scopus citations

Abstract

Autonomic disorders can be the result of autoimmunity. The classic, well-characterized example is autoimmune autonomic ganglionopathy (AAG), in which antibodies against the ganglionic nicotinic acetylcholine receptor impair autonomic transmission, causing autonomic failure, which responds to immunotherapy. However, a number of other autoimmune disorders cause autonomic failure through a variety of mechanisms. In this article, we review autoimmune disorders causing impairment of the peripheral autonomic nervous system (ganglia and nerves), including AAG, other autoimmune autonomic neuropathies, paraneoplastic autonomic neuropathies, and neuromuscular and rheumatologic diseases with autonomic symptomatology. Awareness of primary autoimmune autonomic disorders and the autonomic manifestations of other autoimmune diseases promotes timely diagnosis and appropriate management, including supportive care for unpleasant or dangerous autonomic dysfunction, a search for underlying malignancy when indicated, and the use of immunotherapy when appropriate. A better understanding of the underlying pathophysiology aids in the judicious use and selection of immunotherapy.

Original languageEnglish (US)
Pages (from-to)277-288
Number of pages12
JournalClinical Autonomic Research
Volume29
Issue number3
DOIs
StatePublished - Jun 1 2019

Keywords

  • Autoimmune autonomic ganglionopathy
  • Autoimmune autonomic neuropathy
  • Immunotherapy
  • Paraneoplastic autonomic neuropathy

ASJC Scopus subject areas

  • Endocrine and Autonomic Systems
  • Clinical Neurology

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