Nakane Shunya
Department of Molecular Neurology and Therapeutics, Kumamoto University Hospital.
Rinsho Shinkeigaku. 2019 Dec 25;59(12):783-790. doi: 10.5692/clinicalneurol.cn-001354. Epub 2019 Nov 23.
Autoimmune autonomic ganglionopathy (AAG) is an acquired immune-mediated disorder of widespread autonomic failure. Approximately half of the patients with AAG have the autoantibodies against the neuronal nicotinic acetylcholine receptor (AChR) in autonomic ganglia. These ganglionic AChR antibodies have the potential to mediate the synaptic transmission in sympathetic, parasympathetic, and enteric ganglia. Therefore, seropositive AAG patients exhibit various autonomic symptoms. Extra-autonomic manifestations (coexistence with brain involvement, sensory disturbance, endocrine disorders, autoimmune diseases and tumors) are present in many patients with AAG. The nicotinic AChRs comprise a family of abundantly expressed ligand-gated cation channels found throughout the central and peripheral nervous systems. Moreover, limited manifestations of autoimmune dysautonomia including autoimmune gastrointestinal dysmotility are newly recognized clinical entity. Although combined immunomodulatory therapy is beneficial for almost all patients with AAG, several case reports of some AAG patients with small benefit exist. This review focuses on the recent progress in the clinical approaches of AAG and its related disorders involving the role of autoantibodies and clinical practice.
自身免疫性自主神经节病（AAG）是一种获得性免疫介导的广泛性自主神经功能衰竭疾病。约半数AAG患者存在针对自主神经节中神经元烟碱型乙酰胆碱受体（AChR）的自身抗体。这些神经节AChR抗体有介导交感、副交感和肠神经节突触传递的潜能。因此，血清学阳性的AAG患者表现出各种自主神经症状。许多AAG患者存在自主神经外表现（合并脑受累、感觉障碍、内分泌紊乱、自身免疫性疾病和肿瘤）。烟碱型AChR是一类在中枢和外周神经系统广泛表达的配体门控阳离子通道家族。此外，包括自身免疫性胃肠动力障碍在内的自身免疫性自主神经功能障碍的有限表现是新认识的临床实体。尽管联合免疫调节治疗对几乎所有AAG患者有益，但仍有一些AAG患者获益较小的病例报告。本综述重点关注AAG及其相关疾病临床治疗方法的最新进展，包括自身抗体的作用及临床实践。
