Rare Nephrology News
5 Facts you should know about
Autoimmune autonomic ganglionopathy (AAG)
Autoimmune autonomic ganglionopathy (AAG) is
an acquired immune-mediated disorder that leads to systemic autonomic failure.
Symptoms may include
severe orthostatic hypotension, fainting, constipation, fixed and dilated pupils, urinary retention, and dry mouth and eyes.
In idiopathic AAG, the body's own immune system targets a receptor in the autonomic ganglia, which is part of a peripheral nerve fiber.
If the AAG is paraneoplastic, they have a form of cancer, and their immune system has produced paraneoplastic antibodies in response to the cancer.
1/3 of people with AAG get better without treament
Approximately one third of people with AAG get better without treatment, but the recovery is often incomplete.