Autoimmune Autonomic Ganglionopathy (AAG)


Fact: The cause of AAG is unknown and was discovered by the MAYO Clinic

​What is it?
Autoimmune autonomic ganglionopathy (AAG)
 is rare autoimmune disorder in which the body's immune system mistakenly attacks and damages certain parts of the autonomic nervous system. AAG can impact people of all ages and both sexes. Approximately 100 Americans are diagnosed with AAG per year. AAG is a treatable antibody-mediated disorder of autonomic ganglionic synaptic transmission. Prior names for AAG include acute pandysautonomia, autoimmune autonomic neuropathy and idiopathic subacute autonomic neuropathy.

Cause
The cause of autoimmune autonomic ganglionopathy is not fully understood.An autoimmune component is presumed, as the body's own immune system damages a receptor in the autonomic ganglia (part of the peripheral autonomic nerve fiber). In one to two-thirds of affected individuals, this condition is associated with high titers of ganglionic acetylcholine receptor antibody (g-AchR antibody). About 60% of cases follow an infection or other illness.

Symptoms
The symptoms of autoimmune autonomic ganglionopathy can include:

  • Severe orthostatic hypotension (low blood pressure upon standing) that persists for weeks to years
  • Fainting
  • Constipation and gastrointestinal dysmotility (a condition in which the muscles and nerves of the digestive system do not move food through the digestive tract efficiently)
  • Urinary retention
  • Fixed and dilated pupils
  • Dry mouth and eyes

Some people with autoimmune autonomic ganglionopathy present with POTS-like symptoms.

Treatment
Since autoimmune autonomic ganglionopathy is so rare, no standard treatments have been established.  Experts familiar with this condition often use plasma exchange or total plasmapheresis, intravenous immunoglobulin (IVIG), IV corticosteroids, or immunosuppressive drugs, such as Rituxan to treat the symptoms of the disease. A therapeutic trial for autoimmune autonomic ganglionopathy is currently being conducted by the Autonomic Disorders Consortium.

Want to learn more?
The following organizations and/or websites offer additional information and education about 
Autoimmune Autonomic Ganglionopathy. They are listed in alphabetical order without any preference or prejudice.  Listing these organizations is not a recommendation or referral in any regard for seeking treatment or consultation or support for treatment.   

Dysautonomia International
Lurie Children's, Chicago
NIH
Patients Like Me
​Rare Diseases Clinical Diseases Network

Information on this page taken from:
​​Dysautonomia International
NIH