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Clinical Autonomic Research

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01-02-2018 | Research Article

Seronegative autoimmune autonomic neuropathy: a distinct clinical entity

Authors: Elisabeth P. Golden, Meredith A. Bryarly, Steven Vernino

Published in: Clinical Autonomic Research | Issue 1/2018

Abstract

Purpose

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.

Methods

Retrospective chart review.

Results

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.

Conclusion

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.

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Title: Seronegative autoimmune autonomic neuropathy: a distinct clinical entity
Authors: Elisabeth P. Golden
Meredith A. Bryarly
Steven Vernino
Publication date: 01-02-2018
Publisher: Springer Berlin Heidelberg
Published in: Clinical Autonomic Research / Issue 1/2018
Print ISSN: 0959-9851
Electronic ISSN: 1619-1560
DOI: https://doi.org/10.1007/s10286-017-0493-8

At a glance: The ONWARDS insulin icodec trials

Springer Medicine

Seronegative autoimmune autonomic neuropathy: a distinct clinical entity

Abstract

Purpose

Methods

Results

Conclusion

At a glance: The ONWARDS insulin icodec trials

Springer Medicine

Abstract

Purpose

Methods

Results

Conclusion

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