TY - JOUR
T1 - Autoimmune autonomic neuropathies and ganglionopathies
T2 - epidemiology, pathophysiology, and therapeutic advances
AU - Golden, Elisabeth P.
AU - Vernino, Steven
N1 - Publisher Copyright:
© 2019, Springer-Verlag GmbH Germany, part of Springer Nature.
PY - 2019/6/1
Y1 - 2019/6/1
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
SN - 0959-9851
VL - 29
SP - 277
EP - 288
JO - Clinical Autonomic Research
JF - Clinical Autonomic Research
IS - 3
ER -