National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

Autoimmune autonomic ganglionopathy


Other Names:
Autoimmune Autonomic Neuropathy
Autoimmune autonomic ganglionopathy (AAG) is a rare autoimmune disorder in which the body's immune system mistakenly attacks and damages certain parts of the autonomic nervous system. Signs and symptoms of the condition vary but may include severe orthostatic hypotension (low blood pressure upon standing); fainting; constipation; fixed and dilated pupils; urinary retention; and/or dry mouth and eyes. The exact underlying cause of AAG is poorly understood.[1][2][3] Treatment depends on many factors including the severity of the condition and the signs and symptoms present in each person. Due to the rarity of AAG, there are no standard treatment protocols; however, treatment with plasmapheresis, intravenous (IV) immunoglobulin, corticosteroids or immunosuppressive drugs has been reported with variable success. Approximately one third of affected people may improve spontaneously without treatment, but the recovery is often incomplete.[2][3]
Last updated: 9/2/2015
The symptoms of autoimmune autonomic ganglionopathy can include:[1][2]
  • Severe orthostatic hypotension (low blood pressure upon standing) that persists for weeks to years
  • Fainting
  • Constipation and gastrointestinal dysmotility (a condition in which the muscles and nerves of the digestive system do not move food through the digestive tract efficiently)
  • Urinary retention
  • Fixed and dilated pupils
  • Dry mouth and eyes

Some people with autoimmune autonomic ganglionopathy present with POTS-like symptoms.[2]

Last updated: 1/20/2017
The cause of autoimmune autonomic ganglionopathy is not fully understood.[1] An autoimmune component is presumed, as the body's own immune system damages a receptor in the autonomic ganglia (part of the peripheral autonomic nerve fiber). In one to two-thirds of affected individuals, this condition is associated with high titers of ganglionic acetylcholine receptor antibody (g-AchR antibody).[2][3]. About 60% of cases follow an infection or other illness.[3]
Last updated: 9/2/2015
Since autoimmune autonomic ganglionopathy is so rare, no standard treatments have been established.[1][2][3] 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.[2] A therapeutic trial for autoimmune autonomic ganglionopathy was recently completed by the Autonomic Disorders Consortium.[1]
Last updated: 9/2/2015

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

  • ClinicalTrials.gov lists trials that are related to Autoimmune autonomic ganglionopathy. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

    Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.

Patient Registry

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

Organizations Providing General Support

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

  • Dysautonomia International offers an information page on Autoimmune autonomic ganglionopathy. Please click on the link to access this resource.
  • The Autonomic Disorders Consortium has information about Autoimmune autonomic ganglionopathy for patients and their families. This research consortium is funded by the National Institutes of Health.

In-Depth Information

  • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Autoimmune autonomic ganglionopathy. Click on the link to view a sample search on this topic.

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  1. Autoimmune Autonomic Ganglionopathy. Autonomic Disorders Consortium. http://www.rarediseasesnetwork.org/ARDCRC/patients/learnmore/AAG/. Accessed 9/2/2015.
  2. Autoimmune Autonomic Ganglionopathy Summary. Dysautonomia International. http://www.dysautonomiainternational.org/page.php?ID=124. Accessed 9/2/2015.
  3. Mohini Gurme, MD. Idiopathic Orthostatic Hypotension and other Autonomic Failure Syndromes. Medscape Reference. March 2014; http://emedicine.medscape.com/article/1154266-overview. Accessed 9/2/2015.