Rare Nephrology News
Autoimmune Autonomic Ganglionopathy (AAG) is a very rare form of dysautonomia in which the bodies own immune system damages a receptor in the autonomic ganglia
Age of Onset
5 Facts you should know
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
Approximately one third of people with AAG get better without treatment, but the recovery is often incomplete
Interest over time
Common signs & symptoms
Severe orthostatic hypotension
Low blood pressure upon standing
Constipation and gastrointestinal dysmotility
Fixed and dilated pupils
Dry mouth and eyes
Treatment for autoimmune autonomic ganglionopathy (AAG) is focused on managing the symptoms. Because AAG is so rare, there is no standard treatment. 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. Some people with AAG require a combination of treatments to get better.
Top Clinical Trials
At the time this analysis was conducted, there were no treatments in development for this disease.
Top Treatments in Research
At the time this analysis was conducted, there were no interventional clinical studies being conducted.