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Autoimmune autonomic ganglionopathy | Disease | Overview | Genetic and Rare Diseases Information Center (GARD) – an NCATS Program

Autoimmune autonomic ganglionopathy | Disease | Overview | Genetic and Rare Diseases Information Center (GARD) – an NCATS Program

National Center for Advancing and Translational Sciences

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

02/02/2016 04:30 PM EST

Source: Office of Rare Diseases - NIH
Related MedlinePlus Page: Autonomic Nervous System Disorders

Autoimmune autonomic ganglionopathy (AAG) is rare autoimmune disorder in which the body's immune systemmistakenly 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


  1. Autoimmune Autonomic Ganglionopathy. Autonomic Disorders Consortium. Accessed 9/2/2015.
  2. Autoimmune Autonomic Ganglionopathy Summary. Dysautonomia International. Accessed 9/2/2015.
  3. Mohini Gurme, MD. Idiopathic Orthostatic Hypotension and other Autonomic Failure Syndromes. Medscape Reference. March 2014; Accessed 9/2/2015.
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Basic Information

  • 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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