本研究的目的是报告与免疫检查点抑制剂（ICIs）相关的自主神经失调的临床特征。我们报道了两位患有自身免疫性自主神经节神经病（AAG）的患者，这是由免疫相关的不良事件（irAEs）引起的。我们还对以前报道的ICI治疗期间出现自主神经失调的病例进行了综述。此外，我们使用美国食品和药物管理局不良事件报告系统（FAERS）进行药物监测分析，以调查与ICI相关的自主神经失调。我们所照顾的两位患者，在接受ICI治疗肺癌后发展出了AAG和自身免疫性脑炎。我们全面回顾了13例发表的ICI相关自主神经失调病例（男性：女性=11：2，发病平均年龄为53岁），其中包括AAG（n=3）和自主神经病变（n=10）。其中，七例接受ICI单药治疗，六例接受ICI联合治疗。在13例患者中，有6例自主神经失调出现在ICI开始治疗的一个月内。7例患者出现了体位性低血压，5例出现了尿失禁或尿潴留。除了三例患者外，所有患者都表现出消化系统症状。抗节后乙酰胆碱受体抗体无法检测出来。除了两名患者外，所有患者都接受了免疫调节治疗。三例AAG患者和两例自主神经病变患者对免疫调节治疗有效，但其他患者无效。五名患者死亡，其中三名死于神经学irAE，两名死于癌症。使用FAERS的药物监测分析显示，ipilimumab单药治疗和nivolumab和ipilimumab联合使用，构成了发生自主神经失调的显著风险，与文献综述一致。ICIs可以引起自主神经失调，包括AAG，而自主神经病变是神经学irAE。© 2023作者，独家许可 Springer-Verlag GmbH Germany。

The purpose of this study is to report the clinical characteristics of dysautonomia associated with immune checkpoint inhibitors (ICIs).We reported two patients with autoimmune autonomic ganglionopathy (AAG) occurring as immune-related adverse events (irAEs). We also performed a review of previous case reports presenting dysautonomia during ICI therapy. Moreover, we conducted pharmacovigilance analyses using the US Food and Drug Administration Adverse Events Reporting System (FAERS) to investigate dysautonomia associated with ICI.Two patients in our care developed both AAG and autoimmune encephalitis following ICI therapy for lung cancers. We comprehensively reviewed 13 published cases (M:F = 11:2, mean onset age of 53 years) with ICI-associated dysautonomia including AAG (n = 3) and autonomic neuropathy (n = 10). Of these, ICI monotherapy was performed in seven and combination ICI use in six. In 6 of 13 patients, dysautonomia appeared within one month after the start of ICIs. Orthostatic hypotension was observed in 7 and urinary incontinence or retention in five. All patients except three showed gastrointestinal symptoms. Anti-ganglionic acetylcholine receptor antibodies were undetectable. All but two patients received immune-modulating therapy. Immuno-modulating therapy was effective in three patients with AAG and two patients with autonomic neuropathy, but ineffective in the others. Five patients died, of either the neurological irAE (n = 3) or cancer (n = 2). The pharmacovigilance analyses using FAERS showed that ipilimumab monotherapy and the combination of nivolumab and ipilimumab constituted significant risks for developing dysautonomia, consistent with the review of literature.ICIs can cause dysautonomia including AAG, and autonomic neuropathy is a neurological irAE.© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany.