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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

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ICD-10

N/A

Inheritance

Autosomal dominant

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Autosomal recessive

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Mitochondrial/Multigenic

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X-linked dominant

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X-linked recessive

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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, they 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

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

Autoimmune Autonomic Ganglionopathy (AAG)

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.[1][2][3]

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.