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

2008; Elsevier BV; Volume: 146; Issue: 1-2 Linguagem: Inglês

10.1016/j.autneu.2008.12.002

1872-7484

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

Peripheral Neuropathies and Disorders

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.