Autoimmune dysautonomia / Autoimmune autonomic ganglionopathy / Autoimmune gastrointestinal dysmotility

Summary

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]

(Autoimmune Association, 2023)

Caused by the immune system’s attack on the autonomic nervous system, which regulates non-voluntary bodily functions such as heartbeat, AAG can either be classified as being associated with high levels of g-AchR antibodies (seropositive) or as having no detectable levels of the antibodies (seronegative).

(Global Autoimmune Institute, 2023)

Symptoms

Constipation, urinary retention, fainting, fixated and dilated pupils, dry mouth and eyes, absence of sweating, abdominal pain and vomiting, sexual dysfunction, and very low blood pressure upon standing (severe neurogenic orthostatic hypotension, also known as postural orthostatic tachycardia syndrome (POTS).

(Global Autoimmune Institute, 2023)

Diagnostic Criteria

Under Investigation

Diagnostic Tests

Under Investigation

Human Leukocyte Antigen (HLA) Associations

Under Investigation

Tissue-Type or Cell-Type Attacked

Under Investigation

Attacking Immune Cell Type or Process

Under Investigation

Treatment Modality

Under Investigation

Managing Specialist(s)

Under Investigation

Associated Infections

Under Investigation

Associated Toxins

Under Investigation

Research Authors

Under Investigation

Research Institutions

Under Investigation

Average Time from Symptom Onset to Diagnosis

Under Investigation

Last Updated

January 06, 2023

References

Autoimmune dysautonomia. Autoimmune Association. (2023). Retrieved January 06, 2023, from https://autoimmune.org/disease-information/autoimmune-dysautonomia/

Autoimmune Autonomic Ganglionopathy. Global Autoimmune Institute. (2023). Retrieved January 06, 2023, from https://www.autoimmuneinstitute.org/autoimmune-autonomic-ganglionpathy/