Autoimmune autonomic ganglionopathy with Sjögren's syndrome: Significance of ganglionic acetylcholine receptor antibody and therapeutic approach

Takayuki Kondo; Haruhisa Inoue; Takashi Usui; Tsuneyo Mimori; Hidekazu Tomimoto; Steven Vernino; Ryosuke Takahashi

doi:10.1016/j.autneu.2008.12.002

Autoimmune autonomic ganglionopathy with Sjögren's syndrome: Significance of ganglionic acetylcholine receptor antibody and therapeutic approach

Takayuki Kondo, Haruhisa Inoue, Takashi Usui, Tsuneyo Mimori, Hidekazu Tomimoto, Steven Vernino, Ryosuke Takahashi

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34 Scopus citations

Abstract

Autoimmune autonomic ganglionopathy (AAG) is a disorder defined by antibodies to the nicotinic acetylcholine receptor of the autonomic ganglia. We report two patients with chronically progressing dysautonomia with Sjögren's syndrome (SS). The first case showed elevated titer of ganglionic acetylcholine receptor (AChR) antibody and improved with oral intake of prednisolone. In contrast, the second case showed no elevation of ganglionic AChR antibody titer and had poor response to immunomodulatory therapy. These two cases indicate that chronic AAG may be treatable by immunomodulatory therapy, and have relevance to SS.

Original language	English (US)
Pages (from-to)	33-35
Number of pages	3
Journal	Autonomic Neuroscience: Basic and Clinical
Volume	146
Issue number	1-2
DOIs	https://doi.org/10.1016/j.autneu.2008.12.002
State	Published - Mar 12 2009

Keywords

Autoimmune autonomic ganglionopathy
Ganglionic acetylcholine receptor antibody
Prednisolone
Sjögren's syndrome

ASJC Scopus subject areas

Endocrine and Autonomic Systems
Clinical Neurology
Cellular and Molecular Neuroscience

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10.1016/j.autneu.2008.12.002

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abstract = "Autoimmune autonomic ganglionopathy (AAG) is a disorder defined by antibodies to the nicotinic acetylcholine receptor of the autonomic ganglia. We report two patients with chronically progressing dysautonomia with Sj{\"o}gren's syndrome (SS). The first case showed elevated titer of ganglionic acetylcholine receptor (AChR) antibody and improved with oral intake of prednisolone. In contrast, the second case showed no elevation of ganglionic AChR antibody titer and had poor response to immunomodulatory therapy. These two cases indicate that chronic AAG may be treatable by immunomodulatory therapy, and have relevance to SS.",

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AU - Usui, Takashi

AU - Mimori, Tsuneyo

AU - Tomimoto, Hidekazu

AU - Vernino, Steven

AU - Takahashi, Ryosuke

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Autoimmune autonomic ganglionopathy with Sjögren's syndrome: Significance of ganglionic acetylcholine receptor antibody and therapeutic approach

Abstract

Keywords

ASJC Scopus subject areas

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