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Autoimmune Autonomic Ganglionopathy (AAG)

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

Unknown

US Estimated

Unknown

Europe Estimated

Age of Onset

ICD-10

N/A

Inheritance

This condition does not appear to have a clear pattern of inheritance.

Rare View

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

1

Autoimmune autonomic ganglionopathy (AAG) is an acquired immune-mediated disorder that leads to systemic autonomic failure

FACT

2

Symptoms may include severe orthostatic hypotension, fainting, constipation, fixed and dilated pupils, urinary retention, and dry mouth and eyes

FACT

3

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

4

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

5

Approximately one third of people with AAG get better without treatment, but the recovery is often incomplete

Interest over time

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Autoimmune Autonomic Ganglionopathy (AAG) is also known as...

Autoimmune Autonomic Ganglionopathy (AAG) is also known as:

  • AAG
  • Autoimmune Autonomic Neuropathy

What’s your Rare IQ?

Which of these symptoms is not considered a hallmark of Autoimmune Autonomic Ganglionopathy (AAG)?

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.