Imrich Richard, Vernino Steven, Eldadah Basil A, Holmes Courtney, Goldstein David S
Clinical Neurocardiology Section, National Institute of Neurological Disorders and Stroke, Bethesda, MD 20892-1620, USA.
Clin Auton Res. 2009 Aug;19(4):259-62. doi: 10.1007/s10286-009-0012-7. Epub 2009 Apr 28.
This case report describes successful treatment of autoimmune autonomic ganglionopathy (AAG) in a 74-year-old woman by total plasma exchanges (PLEX) and rituximab. Two series of PLEX temporarily, but dramatically improved orthostatic intolerance and hypotension and baroreflex function, in a manner inversely related to ganglionic neuronal nicotinic receptor antibody titer. After rituximab treatment, the antibody titer was decreased modestly but persistently, and the patient had symptomatic and clinical laboratory evidence of continued benefit for at least 10 months, supporting the autoimmune pathogenesis of AAG.
本病例报告描述了一名74岁女性患者通过全血浆置换(PLEX)和利妥昔单抗成功治疗自身免疫性自主神经节病(AAG)的过程。两疗程的PLEX暂时但显著改善了直立不耐受、低血压及压力反射功能,改善程度与神经节神经元烟碱受体抗体滴度呈负相关。利妥昔单抗治疗后,抗体滴度虽有适度但持续的下降,且患者至少10个月内有症状改善及临床实验室证据表明持续获益,支持了AAG的自身免疫发病机制。