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

Elisabeth P. Golden, Steven Vernino

Research output: Contribution to journalReview article

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)
JournalClinical Autonomic Research
DOIs
StatePublished - Jan 1 2019

Fingerprint

Immunotherapy
Epidemiology
Paraneoplastic Polyneuropathy
Neuromuscular Diseases
Autonomic Nervous System
Peripheral Nervous System
Nicotinic Receptors
Autoimmunity
Ganglia
Autoimmune Diseases
Therapeutics
Antibodies
Neoplasms

Keywords

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

ASJC Scopus subject areas

  • Endocrine and Autonomic Systems
  • Clinical Neurology

Cite this

@article{f98c8cd104da472a91ed1372ac4543f0,
title = "Autoimmune autonomic neuropathies and ganglionopathies: epidemiology, pathophysiology, and therapeutic advances",
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.",
keywords = "Autoimmune autonomic ganglionopathy, Autoimmune autonomic neuropathy, Immunotherapy, Paraneoplastic autonomic neuropathy",
author = "Golden, {Elisabeth P.} and Steven Vernino",
year = "2019",
month = "1",
day = "1",
doi = "10.1007/s10286-019-00611-1",
language = "English (US)",
journal = "Clinical Autonomic Research",
issn = "0959-9851",
publisher = "D. Steinkopff-Verlag",

}

TY - JOUR

T1 - Autoimmune autonomic neuropathies and ganglionopathies

T2 - epidemiology, pathophysiology, and therapeutic advances

AU - Golden, Elisabeth P.

AU - Vernino, Steven

PY - 2019/1/1

Y1 - 2019/1/1

N2 - 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.

AB - 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.

KW - Autoimmune autonomic ganglionopathy

KW - Autoimmune autonomic neuropathy

KW - Immunotherapy

KW - Paraneoplastic autonomic neuropathy

UR - http://www.scopus.com/inward/record.url?scp=85066036338&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85066036338&partnerID=8YFLogxK

U2 - 10.1007/s10286-019-00611-1

DO - 10.1007/s10286-019-00611-1

M3 - Review article

C2 - 31093868

AN - SCOPUS:85066036338

JO - Clinical Autonomic Research

JF - Clinical Autonomic Research

SN - 0959-9851

ER -