自身免疫性自主神经节病的诊治新进展

Diagnosis and Treatment Update on Autoimmune Autonomic Gangliopathy

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 gangliopathy （AAG） is an antibody-mediated disease specifically affecting autonomic nervous system, which is usually of acute or subacute onset. The clinical features include orthostatic syncope, anhidrosis, bladder dysfunction, gastrointestinal dysmotility and erectile dysfunction. Physical examination and laboratory tests indicated non-specific dysfunction of autonomic nervous system. If paraneoplastic syndrome, multiple system atrophy or diabetes can be exclude, patients with autonomic dysfunction can be diagnosed with AAG. Increase of titer of ganglionic AChR antibody in the patient＇ s sera confirms the diagnosis ofAAG. Immunosuppressive therapy is usually required to treat AAG. Steroids is used for the mild cases and intravenous immunoglobulin, plasma exchange and combined immunosuppressive therapy are sometimes required for more refractory cases.