Autoimmune autonomic disorders (autoimmune dysautonomia)

Overview

Autoimmune autonomic disorders (also called autoimmune dysautonomia) are a group of conditions in which the immune system mistakenly attacks components of the autonomic nervous system, disrupting the automatic functions that control blood pressure, heart rate, digestion, sweating, bladder function, and other involuntary body processes.

Some autoimmune autonomic disorders primarily target the autonomic nervous system itself, while others occur as part of broader autoimmune, neurological, or paraneoplastic disorders with prominent autonomic involvement. Although individually rare, many respond to immunotherapy when recognized and treated early.

Autoimmune autonomic disorders include:

Autoimmune autonomic ganglionopathy (AAG)

Autoimmune gastrointestinal dysmotility (AGID)

Immune-mediated sensory and autonomic neuropathies

Paraneoplastic autonomic neuropathies

Acquired idiopathic generalized anhidrosis (AIGA)

Limited autoimmune cholinergic neuropathies

Autonomic dysfunction can also occur as part of other autoimmune and immune-mediated diseases, including:

Sjögren disease

Lambert-Eaton myasthenic syndrome

Guillain-Barré syndrome (GBS)

Anti-NMDA receptor encephalitis

Systemic lupus erythematosus

Rheumatoid arthritis

Postural orthostatic tachycardia syndrome (POTS)

Scleroderma (systemic sclerosis)

Anti-IgLON5 disease

Anti-LGI1 and CASPR2 antibody disorders (including Morvan syndrome)

DPPX-associated encephalitis

Sources

  1. Sources
    1. Mohapatra, P., Agarwal, A., Radhakrishnan, D. M., Srivastava, A. K., & Garg, D. (2024). Decoding Autoimmune Autonomic Disorders: A Less-Recognized Overlap. Annals of Indian Academy of Neurology27(5), 482–492. https://doi.org/10.4103/aian.aian_394_24

    2. Vernino S. (2020). Autoimmune Autonomic Disorders. Continuum (Minneapolis, Minn.)26(1), 44–57. https://doi.org/10.1212/CON.0000000000000812

    3. Nakane, S., Koike, H., Hayashi, T., & Nakatsuji, Y. (2024). Autoimmune Autonomic Neuropathy: From Pathogenesis to Diagnosis. International journal of molecular sciences25(4), 2296. https://doi.org/10.3390/ijms25042296

    4. Kaur, D., Tiwana, H., Stino, A., & Sandroni, P. (2021). Autonomic neuropathies. Muscle & nerve63(1), 10–21. https://doi.org/10.1002/mus.27048