Autoimmune autonomic ganglionopathy (AAG) is a rare type of autonomic neuropathy. In AAG, your immune system attacks your autonomic nervous system. This system controls involuntary body functions, like your heart rate, blood pressure or digestion. If you have AAG, you might faint or feel dizzy when you stand up.
Autoimmune autonomic ganglionopathy (AAG) is an autoimmune disease where your immune system attacks your autonomic nervous system by mistake. The autonomic nervous system is part of your peripheral nervous system. It controls specific involuntary body processes, such as your breathing, blood pressure or heart rate.
AAG is a type of autonomic neuropathy or dysautonomia. Autonomic neuropathies and dysautonomias are disorders of your autonomic nervous system.
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AAG is extremely rare. In the United States, doctors diagnose about 100 people with AAG each year.
In AAG, your immune system damages a specific receptor in your autonomic ganglia. The autonomic ganglia are clusters of nerve cells throughout your autonomic nervous system.
These nerve cell clusters send and receive signals that control your reflexes and other involuntary body functions. Involuntary functions are functions you can’t consciously control, from your heartbeat to the way your other organs work.
When you have damaged receptors in your autonomic ganglia, your body doesn’t process these signals correctly. As a result, some of your automatic body functions, such as blood pressure regulation, may not work properly.
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Experts don’t know exactly what causes autoimmune autonomic ganglionopathy. About 3 in 5 people with AAG develop it after getting another illness or infection.
Some people have paraneoplastic AAG. Paraneoplastic AAG occurs when your immune system makes certain antibodies in response to cancer. As with other forms of AAG, these antibodies attack the autonomic nervous system.
Symptoms of AAG can vary. You may have:
About 2 in 3 people with AAG have high levels of antibodies called ganglionic acetylcholine receptor antibodies (g-AChR antibodies). Researchers think that your g-AChR antibody levels correlate to how severe your symptoms are.
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To diagnose AAG, your healthcare provider will ask you questions about your symptoms and perform a clinical exam. Your provider may use tests that check your autonomic nervous system. You may have:
You may also have a blood test to measure your g-AChR antibody levels. High levels of g-AChR antibodies may point to AAG. This blood test isn’t the same as an AChR antibody test, which checks for myasthenia gravis.
AAG is so rare that healthcare providers don’t have standardized treatment for it. Usually, your treatment plan focuses on symptom management.
Your healthcare provider may use a combination of approaches, including:
There’s no guaranteed way to prevent AAG, although the condition is rare. Some factors, such as a cancer diagnosis, may increase your risk. If you have had cancer, you can increase your overall well-being by adopting healthy lifestyle choices such as:
AAG isn’t curable. But many people successfully manage symptoms long-term. For about 1 in 3 people, AAG symptoms improve without treatment. Most people with AAG deal with some symptoms for the rest of their lives.
What else should I ask my healthcare provider?
You may want to ask your healthcare provider:
AAG is unique because it is both an autoimmune condition and a type of autonomic neuropathy.
Some other autoimmune conditions can increase your chances of developing dysautonomia. These conditions include:
A note from Cleveland Clinic
Autoimmune autonomic ganglionopathy is a rare disorder that affects your nervous system. In AAG, your immune system attacks your autonomic nervous system. You may experience fainting, low blood pressure upon standing or gastrointestinal symptoms. Your treatment usually includes many approaches, such as immunosuppressant medications or plasmapheresis. Although there’s no cure for AAG, many people successfully manage symptoms long-term.
Last reviewed on 04/18/2022.
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Cleveland Clinic is a non-profit academic medical center. Advertising on our site helps support our mission. We do not endorse non-Cleveland Clinic products or services. Policy