Autoimmune autonomic disorders
Autoimmune autonomic disorders
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DOI：
10.1016/b978-0-444-63432-0.00022-0
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发表时间：
2016-01-01
期刊：
影响因子：
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通讯作者：
Benarroch, Eduardo E.
中科院分区：
文献类型：
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作者：
McKeon, Andrew;Benarroch, Eduardo E.
自身免疫性自主神经疾病的发生是由于针对交感神经、副交感神经、肠神经节、自主神经或中枢自主神经通路的免疫反应。一般来说，外周自身免疫性疾病表现为全身性或限制性自主神经衰竭，而中枢自身免疫性疾病主要表现为自主神经亢进。一些自主神经紊乱是全身性的，而另一些在解剖学范围上是有限的，如孤立性胃肠运动障碍。从历史上看，这些疾病很少被认识，并被认为是神经退行性疾病。在过去的20年里，许多自身抗体生物标志物已经被发现，能够识别某些患者是否有自主神经衰竭或多动的自身免疫基础。外周自身免疫性自主神经疾病包括自身免疫性自主神经节病（AAG）、副肿瘤自主神经病变、急性自主神经和感觉神经病变。AAG表现为急性或亚急性发作的全身性或选择性自主神经衰竭。在大约50%的AAG病例中检测到针对神经节型尼古丁乙酰胆碱受体(α 3gAChR) α 3亚基的抗体。其他一些疾病在免疫学上以副肿瘤抗体为特征，这些抗体对癌症具有很高的阳性预测价值，如抗神经元核抗体1型（ANNA-1：抗hu）；其他人仍然是血清阴性。认识到自主神经紊乱的自身免疫基础是很重要的，因为它们的表现是致残的，可能反映了潜在的肿瘤，并且有可能通过对症和免疫治疗的结合来改善。
Autoimmune autonomic disorders occur because of an immune response directed against sympathetic, parasympathetic, and enteric ganglia, autonomic nerves, or central autonomic pathways. In general, peripheral autoimmune disorders manifest with either generalized or restricted autonomic failure, whereas central autoimmune disorders manifest primarily with autonomic hyperactivity. Some autonomic disorders are generalized, and others are limited in their anatomic extent, e.g., isolated gastrointestinal dysmotility. Historically, these disorders were poorly recognized, and thought to be neurodegenerative. Over the last 20 years a number of autoantibody biomarkers have been discovered that have enabled the identification of certain patients as having an autoimmune basis for either autonomic failure or hyperactivity. Peripheral autoimmune autonomic disorders include autoimmune autonomic ganglionopathy (AAG), paraneoplastic autonomic neuropathy, and acute autonomic and sensory neuropathy. AAG manifests with acute or subacute onset of generalized or selective autonomic failure. Antibody targeting the alpha 3 subunit of the ganglionic-type nicotinic acetylcholine receptor (alpha 3gAChR) is detected in approximately 50% of cases of AAG. Some other disorders are characterized immunologically by paraneoplastic antibodies with a high positive predictive value for cancer, such as antineuronal nuclear antibody, type 1 (ANNA-1: anti-Hu); others still are seronegative. Recognition of an autoimmune basis for autonomic disorders is important, as their manifestations are disabling, may reflect an underlying neoplasm, and have the potential to improve with a combination of symptomatic and immune therapies.