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自身免疫性自主神经节病(AAG)为一种与自身抗体相关、由免疫介导的自主神经功能障碍疾病。多为急性或亚急性起病,常见临床表现为体位性昏厥、汗液分泌异常、膀胱功能障碍、胃肠动力下降与性功能障碍等植物神经受累等。体格检查和实验室检查提示相应的自主神经功能异常,但缺乏特异性。如排除副肿瘤性、多脏器萎缩或糖尿病等所致的自主神经功能障碍,可诊断为AAG;如患者血清中抗自主神经节乙酰胆碱受体抗体滴度升高则可明确诊断。临床上以免疫抑制治疗为主,主要应用静脉注射丙种球蛋白(IVIG)、血浆置换、激素、免疫抑制剂等免疫调节治疗,但剂量与疗程目前只能依靠医生个人经验及患者临床缓解情况而决定。如疗效不佳则用IVIG、血浆置换与免疫抑制剂联合治疗。
Autoimmune Autonomic Ganglioneopathy (AAG) is an autoantibody-related, autoimmune-mediated autonomic dysfunction disease. Mostly acute or subacute onset, the common clinical manifestations of fainting, abnormal sweat secretion, bladder dysfunction, decreased gastrointestinal motility and sexual dysfunction and other autonomic involvement. Physical examination and laboratory tests suggest a corresponding autonomic dysfunction, but lack specificity. Such as the exclusion of paraneoplastic, multiple organ atrophy or diabetes due to autonomic dysfunction, can be diagnosed as AAG; such as serum anti-autonomic ganglia acetylcholine receptor antibody titers can be clearly diagnosed. Clinically, immunosuppressive therapy is mainly used for intravenous immunoglobulin (IVIG), plasma exchange, hormone, immunosuppressive agents and other immunomodulatory treatment, but the dose and course of treatment can only rely on the doctor’s personal experience and clinical response to the patient’s condition Decided. Such as poor efficacy with IVIG, plasma exchange and immunosuppressive agents combined treatment.