Autoimmune autonomic ganglionopathy (AAG) is a condition in which the body’s immune system mistakenly attacks and damages certain parts of the autonomic nervous system. AAG may be divided into two different types based on the presence of specific types of cells in the blood that normally fight infection (antibodies). Signs and symptoms of AAG usually begin in adulthood and are different from person to person. Symptoms may include severe orthostatic hypotension (low blood pressure upon standing), fainting, constipation, fixed and dilated pupils, urinary retention, and dry mouth and eyes. The underlying cause of AAG is poorly understood. Diagnosis is based on the symptoms, clinical exam, and specialized laboratory tests. 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 is no standard treatment. Treatment options include plasmapheresis, intravenous (IV) immunoglobulin, corticosteroids, or immunosuppressive drugs. Approximately one third of people with AAG get better without treatment, but the recovery is often incomplete.[1][2][3]
Symptoms
- 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.[5]
