Autoimmune Autonomic Ganglionopathy (AAG)

What is autoimmune autonomic ganglionopathy (AAG)?

Autoimmune autonomic ganglionopathy, or AAG for short, is a rare autoimmune disorder characterized by the failure of the autonomic nervous system; specifically, antibodies are incorrectly directed against a specific receptor in their own autonomic ganglia cells.

What causes autoimmune autonomic ganglionopathy (AAG)?

The cause of AAG is not fully understood. In one- to two-thirds of affected individuals, this condition is associated with high titers of ganglionic acetylcholine receptor antibody (g-AchR antibody). In addition, about 60% of cases follow an infection or other illness.

What are the symptoms of autoimmune autonomic ganglionopathy (AAG)?

The following are some of the common signs and symptoms of AAG:

Severe orthostatic hypotension
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
Other POTS-like symptoms

How is autoimmune autonomic ganglionopathy (AAG) diagnosed?

After a thorough clinical evaluation and a detailed patient and family history, tests to reach a diagnosis of AAG include:

Tilt Table Test (TTT)
Thermoregulatory sweat test (TST)
Quantitative sudomotor autonomic reflex testing (QSART)
Blood panels and tests

What are the available treatments for autoimmune autonomic ganglionopathy (AAG)?

To date, no treatment specifically for AAG exists. However, treatment for the symptoms of the disease may include:

Plasma exchange or total plasmapheresis
Intravenous immunoglobulin (IVIG)
IV corticosteroids
Immunosuppressive drugs

Where can I find out more about autoimmune autonomic ganglionopathy (AAG)?