Rare Pulmonology 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
Prevelance
Age of Onset
ICD-10
N/A
Inheritance
Autosomal dominant
Autosomal recessive
Mitochondrial/Multigenic
X-linked dominant
X-linked recessive
5 Facts you should know
FACT
Autoimmune autonomic ganglionopathy (AAG) is an acquired immune-mediated disorder that leads to systemic autonomic failure
FACT
Symptoms may include severe orthostatic hypotension, fainting, constipation, fixed and dilated pupils, urinary retention, and dry mouth and eyes
FACT
In idiopathic AAG, the body's own immune system targets a receptor in the autonomic ganglia, which is part of a peripheral nerve fiber
FACT
If the AAG is paraneoplastic, they have a form of cancer, and their immune system has produced paraneoplastic antibodies in response to the cancer
FACT
Approximately one third of people with AAG get better without treatment, but the recovery is often incomplete
Interest over time
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Common signs & symptoms
Severe orthostatic hypotension
Low blood pressure upon standing
Fainting
Constipation and gastrointestinal dysmotility
Urinary retention
Fixed and dilated pupils
Dry mouth and eyes
Current treatments
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.[1][2][3]
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.