Autoimmune Autonomic Neuropathy

Understanding Autoimmune Autonomic Neuropathy

Autoimmune autonomic neuropathy, also known as autoimmune autonomic ganglionopathy, is believed to be an autoimmune disorder. An autoimmune disorder develops when the immune system malfunctions and creates antibodies that attack and cause damage to a part of the body. In autoimmune autonomic neuropathy, a specific receptor in the autonomic ganglia, which are clusters of nerve cells throughout the autonomic nervous system, is attacked. This attack on the autonomic ganglia results in problems with reflexes and other involuntary body functions. Autoimmune autonomic neuropathy is extremely rare. In the United States, about 100 people are diagnosed with autoimmune autonomic neuropathy each year.

Symptoms of Autoimmune Autonomic Neuropathy

Signs and symptoms of autoimmune autonomic neuropathy 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.

Diagnosing Autoimmune Autonomic Neuropathy

The underlying cause of autoimmune autonomic neuropathy is poorly understood. Diagnosis is based on symptoms, clinical exam, and specialized laboratory tests that check the autonomic nervous system such as gastrointestinal tests (to check gastric emptying and digestion), quantitative sudomotor axon reflex test [QSART] (to measure sweat glands function), thermoregulatory sweat test (to find the cause of decreased or increased sweating), tilt table test (to check heart rate and blood pressure when changing positions) and urinalysis (to check bladder function).

Treatment of Autoimmune Autonomic Neuropathy

Treatment depends on many factors including the severity and symptoms. Because it is so rare, there is no standard treatment. About one third of people with autoimmune autonomic neuropathy get better without treatment, but the recovery is often incomplete. Treatment options include plasmapheresis, immune globulin therapy administered into the blood stream intravenously (IVIG), corticosteroids, or immunosuppressive drugs. Plasmapheresis (also known as apheresis, plasma exchange, or “plex”) is a medical procedure where a device separates whole blood into the cellular components and plasma. The plasma is then discarded and replaced with a colloid fluid, combined back with the cellular components, and returned to the same patient.

References:

• Autoimmune Autonomic Ganglionopathy | National Organization for Rare Disorders
• Autoimmune Autonomic Ganglionopathy | Cleveland Clinic