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自身免疫性自主神经节病患者接受免疫球蛋白G免疫吸附治疗后的长期心血管自主神经及临床变化

Long-term cardiovascular autonomic and clinical changes after immunoglobulin G immunoadsorption therapy in autoimmune autonomic ganglionopathy.

作者信息

Barbic Franca, Dipaola Franca, Andreetta Francesca, Brunetta Enrico, Dalla Vecchia Laura, Mantegazza Renato, Furlan Raffaello, Antozzi Carlo

机构信息

aInternal Medicine 4, Humanitas Clinical and Research Center, Milano bNeuroimmunology and Neuromuscular Diseases Unit, I.R.C.C.S. Istituto Neurologico 'C. Besta' cI.R.C.C.S Istituti Clinici Scientifici Maugeri dHumanitas University, Rozzano, Milan, Italy.

出版信息

J Hypertens. 2017 Jul;35(7):1513-1520. doi: 10.1097/HJH.0000000000001355.

Abstract

: A 63-year-old male was diagnosed with autoimmune autonomic ganglionopathy based on the finding of plasma antibodies to the nicotinic acetylcholine receptor (nAChR) of autonomic ganglia. He complained of mouth and eye dryness, dysphagia, severe constipation, erectile dysfunction, urgency, frequent urination, habitual orthostatic syncope and presyncope. A remarkable symptomatic orthostatic hypotension without changes in heart rate was present. We here describe the 3-year time course of the changes in spectral indices of cardiovascular autonomic control LF/HF and LFSAP, dysautonomia symptoms intensity and anti-nAChR antibodies following repetitive selective immunoadsorptions. During the follow-up, the reduction of anti-nAChR antibodies produced by immunoadsorption was associated with a diminished orthostatic hypotension, a restored capability to increase LF/HF, LFSAP and norepinephrine in upright position, a decline in the intensity of autonomic symptoms and an improvement of life quality. Spectral parameters LF/HF and LFSAP may represent noninvasive, low-cost biomarkers suitable for autoimmune autonomic ganglionopathy patients' clinical follow-up.

摘要

一名63岁男性基于发现针对自主神经节烟碱型乙酰胆碱受体(nAChR)的血浆抗体而被诊断为自身免疫性自主神经节病。他主诉口干、眼干、吞咽困难、严重便秘、勃起功能障碍、尿急、尿频、习惯性直立性晕厥和晕厥前状态。存在显著的症状性直立性低血压,心率无变化。我们在此描述重复选择性免疫吸附后心血管自主神经控制的频谱指数LF/HF和LFSAP、自主神经功能障碍症状强度以及抗nAChR抗体变化的3年时间进程。在随访期间,免疫吸附产生的抗nAChR抗体减少与直立性低血压减轻、直立位时LF/HF、LFSAP和去甲肾上腺素增加能力恢复、自主神经症状强度下降以及生活质量改善相关。频谱参数LF/HF和LFSAP可能代表适合自身免疫性自主神经节病患者临床随访的无创、低成本生物标志物。

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