Autoimmune Autonomic Ganglionpathy (AAG)

 

Overview

Caused by the immune system’s attack on the autonomic nervous system, which regulates non-voluntary bodily functions such as heartbeat, AAG can either be classified as being associated with high levels of g-AchR antibodies (seropositive) or as having no detectable levels of the antibodies (seronegative).

Common Symptoms

Constipation, urinary retention, fainting, fixated and dilated pupils, dry mouth and eyes, absence of sweating, abdominal pain and vomiting, sexual dysfunction, and very low blood pressure upon standing (severe neurogenic orthostatic hypotension, also known as postural orthostatic tachycardia syndrome (POTS).

Coexisting Diseases and Conditions

Sjogren’s syndrome, systemic lupus erythematosus, rheumatoid arthritis, and mixed connective tissue diseases.

Risk Factors and Prevalence

AAG is most likely to occur between 50-70 years of age, and while it affects both sexes, it is more prevalent in females.

Sources

  1. Article Sources and Footnotes

    1. Autoimmune autonomic ganglionopathy | Genetic and Rare Diseases Information Center (GARD) – an NCATS Program. (n.d.). Https://Rarediseases.Info.Nih.Gov/Diseases/11917/Autoimmune-Autonomic-Ganglionopathy. Retrieved July 12, 2021, from https://rarediseases.info.nih.gov/diseases/11917/autoimmune-autonomic-ganglionopathy

    2. Autonomic Disorder Information. (n.d.). Https://Www.Rarediseasesnetwork.Org/Cms/Autonomic/Learn-More/Disorder-Definitions. Retrieved July 12, 2021, from https://www.rarediseasesnetwork.org/cms/autonomic/Learn-More/Disorder-Definitions

    3. Golden, E. P., & Vernino, S. (2019). Autoimmune autonomic neuropathies and ganglionopathies: Epidemiology, pathophysiology, and therapeutic advances. Clinical Autonomic Research, 29(3), 277–288. https://doi.org/10.1007/s10286-019-00611-1

    4. Nakane, S., Higuchi, O., Koga, M., Kanda, T., Murata, K., Suzuki, T., Kurono, H., Kunimoto, M., Kaida, K., Mukaino, A., Sakai, W., Maeda, Y., & Matsuo, H. (2015). Clinical Features of Autoimmune Autonomic Ganglionopathy and the Detection of Subunit-Specific Autoantibodies to the Ganglionic Acetylcholine Receptor in Japanese Patients. PLoS ONE, 10(3), e0118312. https://doi.org/10.1371/journal.pone.0118312