Clinical features of autoimmune autonomic ganglionopathy and the detection of subunit-specific autoantibodies to the ganglionic acetylcholine receptor in Japanese patients.
Clinical features of autoimmune autonomic ganglionopathy and the detection of subunit-specific autoantibodies to the ganglionic acetylcholine receptor in Japanese patients.
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DOI：
10.1371/journal.pone.0118312
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发表时间：
2015
期刊：
影响因子：
3.7
通讯作者：
Matsuo H
中科院分区：
文献类型：
--
作者：
Nakane S;Higuchi O;Koga M;Kanda T;Murata K;Suzuki T;Kurono H;Kunimoto M;Kaida K;Mukaino A;Sakai W;Maeda Y;Matsuo H
自身免疫性自主神经节病（AAG）是一种罕见的以泛自主神经为特征的获得性通道病，其中神经节烟碱乙酰胆碱受体（gAChR）的自身抗体可能起核心作用。放射免疫沉淀法（RIP）已被用于检测AAG患者血清中gAChR自身抗体。在此，我们开发了荧光素酶免疫沉淀系统（LIPS），基于血清中α3和β4 gAChR亚基的igg来诊断AAG。我们回顾了50例确诊为AAG的日本患者的血清学和临床资料。通过LIPS检测，我们在48%（24/50）患者中检测到抗α3和-β4 gAChR抗体。与血清阴性组相比，血清阳性组的发病模式更为渐进。AAG患者常伴有直立性低血压和上下胃肠道症状，伴或不伴抗gachr。除血清阳性组中有3例患者出现失弛缓症外，血清阳性组与血清阴性组之间自主神经症状的发生无显著差异。此外，我们发现自身免疫性疾病在血清阳性组和内分泌异常作为AAG的偶发并发症的显著过度代表。我们的研究结果表明，LIPS检测是检测AAG患者抗gAChR自身抗体的一种有用的新工具。
Autoimmune autonomic ganglionopathy (AAG) is a rare acquired channelopathy that is characterized by pandysautonomia, in which autoantibodies to ganglionic nicotinic acetylcholine receptors (gAChR) may play a central role. Radioimmunoprecipitation (RIP) assays have been used for the sensitive detection of autoantibodies to gAChR in the serum of patients with AAG. Here, we developed luciferase immunoprecipitation systems (LIPS) to diagnose AAG based on IgGs to both the α3 and β4 gAChR subunits in patient serum. We reviewed the serological and clinical data of 50 Japanese patients who were diagnosed with AAG. With the LIPS testing, we detected anti-α3 and -β4 gAChR antibodies in 48% (24/50) of the patients. A gradual mode of onset was more common in the seropositive group than in the seronegative group. Patients with AAG frequently have orthostatic hypotension and upper and lower gastrointestinal tract symptoms, with or without anti-gAChR. The occurrence of autonomic symptoms was not significantly different between the seropositive and seronegative group, with the exception of achalasia in three patients from the seropositive group. In addition, we found a significant overrepresentation of autoimmune diseases in the seropositive group and endocrinological abnormalities as an occasional complication of AAG. Our results demonstrated that the LIPS assay was a useful novel tool for detecting autoantibodies against gAChR in patients with AAG.
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影响因子：
2.7
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