Seronegative autoimmune autonomic neuropathy: a distinct clinical entity
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Autoimmune autonomic ganglionopathy (AAG) is associated with ganglionic acetylcholine receptor (gAChR) antibodies. We describe a similar but distinct series of patients with autoimmune autonomic failure lacking this antibody.
Retrospective chart review.
Six patients presented with subacute autonomic failure, seronegative for gAChR antibodies. Orthostatic hypotension and gastrointestinal complaints were common. Autonomic testing revealed predominant sympathetic failure and no premature pupillary redilation. All patients had sensory symptoms and/or pain, which was severe in three. Immunotherapy with plasma exchange, intravenous immunoglobulin, and rituximab was ineffective. Three patients responded to intravenous steroids.
In these cases of autoimmune autonomic failure, key differences from seropositive AAG emerge. Testing showed prominent sympathetic (rather than cholinergic) failure, specific pupillary findings of AAG were absent, and sensory symptoms were prominent. AAG responds to antibody-targeted immunotherapy, while these patients responded best to steroids. This seronegative autoimmune autonomic neuropathy is a distinct clinical entity requiring a different treatment approach from AAG.
KeywordsAutoimmune autonomic ganglionopathy Orthostatic hypotension Autonomic neuropathy Autonomic testing Immunotherapy
We would like to thank Samarpita Sengupta, PhD, for assistance with preparation of the manuscript.
Compliance with ethical standards
Conflict of interest
Drs. Golden and Bryarly have no conflict of interest to report. Dr. Vernino has served as a consultant for Athena Diagnostics and Lundbeck and on the speaker bureau for Lundbeck.
This study was approved by the UT Southwestern Institutional Review Board and was therefore performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki and its later amendments. Subjects gave their informed consent prior to their inclusion in the study.
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