Autoimmune Autonomic Ganglionopathy

What is autoimmune autonomic ganglionopathy?

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.

How common is autoimmune autonomic ganglionopathy?

AAG is extremely rare. In the United States, doctors diagnose about 100 people with AAG each year.

How does autoimmune autonomic ganglionopathy affect my body?

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.

What causes autoimmune autonomic ganglionopathy?

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.

What are the symptoms of autoimmune autonomic ganglionopathy?

Symptoms of AAG can vary. You may have:

• Constipation.
• Dilated pupils (Adie’s pupils).
• Dry mouth or dry eyes.
• Fainting (syncope).
• Low blood pressure when you move to stand (neurogenic orthostatic hypotension).
• Urinary retention.

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.

How is autoimmune autonomic ganglionopathy diagnosed?

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:

• Gastrointestinal tests, such as gastric emptying, to check your digestion.
• Quantitative sudomotor axon reflex test (QSART) to measure how your sweat glands work.
• Thermoregulatory sweat test to find the cause of decreased or increased sweating.
• Tilt table test to check your heart rate and blood pressure when changing positions.
• Urinalysis to check your bladder function.

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.

How is autoimmune autonomic ganglionopathy treated?

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:

• Plasma exchange (plasmapheresis): A process of drawing blood, removing harmful cells from plasma and returning blood to your body.
• Intravenous immunoglobulin (IVIG) therapy: Giving your body extra immunoglobulin, a part of plasma with disease-fighting properties.
• Intravenous corticosteroids: Taking high doses of inflammation-reducing steroids, such as methylprednisolone (Solu-Medrol®) or hydrocortisone (Solu-Cortef®), through an IV.
• Immunosuppressive medications: Immunosuppressants such as rituximab (Rituxan®) that reduce your immune system’s effects on healthy body systems.

How can I prevent autoimmune autonomic ganglionopathy?

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:

• Aerobically exercising.
• Adopting a high-fluid, high-salt diet to help regulate your blood pressure if you have dysautonomia.
• Managing stress with healthy strategies like exercising, talking with a friend or journaling.
• Sleeping well for at least seven to eight hours nightly.
• Wearing compression garments to increase circulation and reduce swelling.

Is autoimmune autonomic ganglionopathy curable?

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:

• What is the most likely cause of my symptoms?
• What tests do I need to diagnose AAG?
• What are the treatment options for AAG?
• What are the chances that AAG symptoms will improve without treatment?
• What lifestyle changes should I make to improve my overall health?

Which autoimmune conditions are associated with autonomic neuropathy?

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:

• Celiac disease.
• Guillain-Barré syndrome.
• Lupus (systemic lupus erythematosus).
• Rheumatoid arthritis.
• Sjögren’s syndrome.

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.