Nakane Shunya, Watari Mari, Ando Yukio
Department of Neurology, Graduate School of Medical Sciences, Kumamoto University.
Brain Nerve. 2018 Apr;70(4):383-393. doi: 10.11477/mf.1416201011.
The clinical associations of the anti-ganglionic acetylcholine receptor (gAChR) have not yet been described fully. It is not known whether central nervous system (CNS) involvement and endocrine disorders are the extra-autonomic features of autoimmune autonomic ganglionopathy (AAG), or whether it is related to circulating anti-gAChR antibodies (Abs). The present study prospectively identified 123 Abs-positive AAG patients in Japan and collated their clinical features, investigations, and immunotherapy responses. Luciferase immunoprecipitation systems were used to detect anti-α3 and -β4 gAChR Abs. A gradual mode of onset was more common among the 123 seropositive AAG patients examined. Patients with AAG demonstrated widespread autonomic dysfunction. In particular, orthostatic hypotension and lower gastrointestinal tract dysfunction were frequently observed. Approximately 80% of patients with seropositive AAG exhibited extra-autonomic manifestations, including CNS involvement, endocrine disorders, other autoimmune disease, and tumors. Additionally, the majority exhibited a marked improvement in clinical status and the levels of anti-gAChR Abs with immunotherapy. CNS involvement and endocrine disorders were frequent among the seropositive patients with AAG, indicating that seropositivity for AAG may be associated with underlying conditions such as autoimmune diseases or tumors.
抗神经节乙酰胆碱受体（gAChR）的临床关联尚未得到充分描述。目前尚不清楚中枢神经系统（CNS）受累和内分泌紊乱是否是自身免疫性自主神经节病（AAG）的自主神经外特征，或者是否与循环抗gAChR抗体（Abs）有关。本研究前瞻性地确定了日本123例抗gAChR抗体阳性的AAG患者，并整理了他们的临床特征、检查结果和免疫治疗反应。采用荧光素酶免疫沉淀系统检测抗α3和抗β4 gAChR抗体。在所检查的123例血清阳性AAG患者中，起病逐渐的模式更为常见。AAG患者表现出广泛的自主神经功能障碍。特别是，直立性低血压和下消化道功能障碍经常被观察到。大约80%的血清阳性AAG患者表现出自主神经外表现，包括CNS受累、内分泌紊乱、其他自身免疫性疾病和肿瘤。此外，大多数患者在免疫治疗后临床状况和抗gAChR抗体水平有明显改善。CNS受累和内分泌紊乱在血清阳性AAG患者中很常见，这表明AAG血清阳性可能与自身免疫性疾病或肿瘤等潜在疾病有关。
