Autoimmune autonomic ganglionopathy with Sjogren's syndrome: Significance of ganglionic acetylcholine receptor antibody and therapeutic approach
Autoimmune autonomic ganglionopathy with Sjogren's syndrome: Significance of ganglionic acetylcholine receptor antibody and therapeutic approach
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DOI：
10.1016/j.autneu.2008.12.002
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发表时间：
2009-03-12
影响因子：
2.7
通讯作者：
Takahashi, Ryosuke
中科院分区：
文献类型：
--
作者：
Kondo, Takayuki;Inoue, Haruhisa;Takahashi, Ryosuke
自身免疫性自主神经节病 (AAG) 是一种由自主神经节烟碱乙酰胆碱受体抗体定义的疾病。我们报告了两名患有慢性进展性自主神经功能障碍并伴有干燥综合征（SS）的患者。第一个病例显示神经节乙酰胆碱受体（AChR）抗体滴度升高，并通过口服泼尼松龙得到改善。相比之下，第二个病例的神经节AChR抗体滴度没有升高，并且对免疫调节治疗的反应较差。这两个病例表明慢性 AAG 可以通过免疫调节治疗来治疗，并且与 SS 相关。 (C) 2008 Elsevier B.V. 保留所有权利。
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 Sjogren'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. (C) 2008 Elsevier B.V. All rights reserved.