Autoantibodies to ganglionic acetylcholine receptors in autoimmune autonomic neuropathies
Autoantibodies to ganglionic acetylcholine receptors in autoimmune autonomic neuropathies
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DOI：
10.1056/nejm200009213431204
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发表时间：
2000-09-21
影响因子：
158.5
通讯作者：
Lennon, VA
中科院分区：
文献类型：
--
作者：
Vernino, S;Low, PA;Lennon, VA
背景：特发性自主神经病是一种严重的亚急性疾病，推测具有自身免疫基础。它与可能伴随肺癌或其他肿瘤的亚急性自主神经病变没有区别。自主神经节中烟碱乙酰胆碱受体特异性的自身抗体具有潜在致病性，并且可以作为各种形式的自身免疫性自主神经病的血清学标志物。方法：我们测试了来自 157 名患有各种类型自主神经功能障碍的患者的血清。使用碘 125 标记的皮巴替丁和溶解的人神经母细胞瘤乙酰胆碱受体进行免疫沉淀测定，以检测结合或阻断神经节受体的自身抗体。结果：在 46 名特发性或副肿瘤性自主神经病患者中，有 19 名患者发现了神经节受体结合抗体（41 %），67 名患有姿势性心动过速综合征、特发性胃肠运动障碍或糖尿病自主神经病变的患者中有 6 名（9%），而 44 名患有其他自主神经疾病的患者则没有。高水平的结合抗体与更严重的自主神经功能障碍（包括强直性瞳孔的存在）相关。在临床改善的患者中，这些抗体的水平下降。所有七名具有神经节受体阻断抗体的患者均具有神经节受体结合抗体，并患有特发性或副肿瘤性自主神经病。 结论：结合或阻断神经节乙酰胆碱受体的抗体的血清阳性可识别患有各种形式的自身免疫性自主神经病的患者并区分这些疾病 来自其他类型的自主神经功能障碍。高水平的神经节受体抗体与自主神经功能障碍的严重程度之间的正相关性表明，这些抗体在这些类型的神经病中具有致病作用。 （N Engl J Med 2000；343：847-55。）(C) 2000，马萨诸塞州医学会。
Background: Idiopathic autonomic neuropathy is a severe, subacute disorder with a presumed autoimmune basis. It is indistinguishable from the subacute autonomic neuropathy that may accompany lung cancer or other tumors. Autoantibodies specific for nicotinic acetylcholine receptors in the autonomic ganglia are potentially pathogenic and may serve as serologic markers of various forms of autoimmune autonomic neuropathy.Methods: We tested serum from 157 patients with a variety of types of dysautonomia. Immunoprecipitation assays with iodine-125-labeled epibatidine and solubilized human neuroblastoma acetylcholine receptors were used to detect autoantibodies that bound to or blocked ganglionic receptors.Results: Ganglionic-receptor-binding antibodies were found in 19 of 46 patients with idiopathic or paraneoplastic autonomic neuropathy (41 percent), in 6 of 67 patients with postural tachycardia syndrome, idiopathic gastrointestinal dysmotility, or diabetic autonomic neuropathy (9 percent), and in none of 44 patients with other autonomic disorders. High levels of the binding antibodies correlated with more severe autonomic dysfunction (including the presence of tonic pupils). Levels of these antibodies decreased in patients who had clinical improvement. All seven patients with ganglionic-receptor-blocking antibodies had ganglionic-receptor-binding antibodies and had idiopathic or paraneoplastic autonomic neuropathy.Conclusions: Seropositivity for antibodies that bind to or block ganglionic acetylcholine receptors identifies patients with various forms of autoimmune autonomic neuropathy and distinguishes these disorders from other types of dysautonomia. The positive correlation between high levels of ganglionic-receptor antibodies and the severity of autonomic dysfunction suggests that the antibodies have a pathogenic role in these types of neuropathy. (N Engl J Med 2000;343:847-55.) (C) 2000, Massachusetts Medical Society.