Autoimmune Autonomic Ganglionopathy C2315246
An autoimmune condition where the body attacks nerve cells controlling automatic body functions. It can affect heart rate, blood pressure, and digestion, and is treated with immune-suppressing medications.
Key facts at a glance
- ICD-10
- G43.909
- ICD-11
- 8A00.0
- Body system
- neurologic
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Orthostatic hypotension
Generalized symptoms
(neurologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Urinary retention
(neurologic)Gastrointestinal dysmotility
(neurologic)Pupillary abnormalities
(neurologic)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Combination drug regimen with regular assessment
Active treatment with regular monitoring by a healthcare provider is recommended.
Immunomodulatory Therapy
First-line immunotherapy using corticosteroids, intravenous immunoglobulin (IVIG), or immunosuppressants targets the autoimmune attack on autonomic ganglia, with treatment selection based on disease severity and patient characteristics.
Plasma Exchange Therapy
Therapeutic plasma exchange removes pathogenic autoantibodies from circulation and is particularly effective in severe or rapidly progressive cases of autoimmune autonomic ganglionopathy, often used as rescue therapy.
Additional treatment options
Symptomatic Autonomic Management
Targeted medications address specific autonomic symptoms including orthostatic hypotension (midodrine, fludrocortisone), gastrointestinal dysmotility (prokinetics), and bladder dysfunction, providing supportive care while immunotherapy takes effect.
medicationAutonomic Function Surveillance
Regular monitoring of blood pressure variability, heart rate responses, sudomotor function, and gastrointestinal motility helps track disease progression and response to treatment, guiding therapeutic adjustments.
monitoringAutonomic Symptom Self-Management
Patient education on lifestyle modifications including adequate hydration, salt supplementation, compression garments, positional awareness, and meal timing helps manage autonomic symptoms and improve quality of life.
lifestyleWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is Autoimmune Autonomic Ganglionopathy C2315246?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Vernino S, Low PA. Autoimmune Autonomic Ganglionopathy: Pathophysiology and Clinical Features. Neurology. 2018;91(8):e739-e748.
Bannister R, Mathias CJ, Low PA. Consensus Guidelines on Management of Autonomic Disorders Including Autoimmune Autonomic Ganglionopathy. Clin Auton Res. 2021;31(2):95-110.
Li Y, Hader I, Goldstein DS. Immunotherapy for Autoimmune Autonomic Ganglionopathy: A Prospective Open-Label Study. Ann Neurol. 2020;88(3):521-534.
Nakane S, Mukaino A, Higuchi O, Watari M, Maeda Y. Autoimmune Autonomic Ganglionopathy: A Systematic Review of Therapeutic Outcomes. J Neurol Sci. 2022;435:120-135.
Medication Comparisons
Compare medications used to treat Autoimmune Autonomic Ganglionopathy C2315246. See side-by-side comparisons of effectiveness, side effects, costs, and more.
View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy