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Autoimmune Autonomic Ganglionopathy (AAG) is a very rare form of dysautonomia in which the bodies own immune system damages a receptor in the autonomic ganglia
Prevalence
Age of Onset
ICD-10
N/A
Inheritance
This condition does not appear to have a clear pattern of inheritance.
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Autoimmune autonomic ganglionopathy (AAG) is an acquired immune-mediated disorder that leads to autonomic failure. The disorder is associated with autoantibodies to the ganglionic nicotinic acetylcholine receptor (gAChR). AAG is divided into two different types based on the presence of specific types of antibodies. Signs and symptoms of AAG usually begin in adulthood and are different from person to person. Symptoms may include severe orthostatic hypotension (low blood pressure upon standing), fainting, constipation, fixed and dilated pupils, urinary retention, and dry mouth and eyes.
5 Facts you should know
FACT
Autoimmune autonomic ganglionopathy (AAG) is an acquired immune-mediated disorder that leads to systemic autonomic failure
FACT
Symptoms may include severe orthostatic hypotension, fainting, constipation, fixed and dilated pupils, urinary retention, and dry mouth and eyes
FACT
In idiopathic AAG, the body's own immune system targets a receptor in the autonomic ganglia, which is part of a peripheral nerve fiber
FACT
If the AAG is paraneoplastic, patients have a form of cancer, and their immune system has produced paraneoplastic antibodies in response to the cancer
FACT
Approximately one third of people with AAG get better without treatment, but the recovery is often incomplete
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Common signs & symptoms
Severe orthostatic hypotension
Low blood pressure upon standing
Fainting
Constipation and gastrointestinal dysmotility
Urinary retention
Fixed and dilated pupils
Dry mouth and eyes
Current treatments
Treatment for autoimmune autonomic ganglionopathy (AAG) is focused on managing the symptoms. Because AAG is so rare, there is no standard treatment. 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. Some people with AAG require a combination of treatments to get better.
Top Clinical Trials
At the time this analysis was conducted, there were no treatments in development for this disease.
Top Treatments in Research
At the time this analysis was conducted, there were no interventional clinical studies being conducted.