Department of Neurosurgery, Inselspital, Bern University Hospital, Bern, Switzerland.
Department of Neurology, Inselspital, Bern University Hospital, Bern, Switzerland.
Eur J Neurol. 2021 May;28(5):1692-1697. doi: 10.1111/ene.14711. Epub 2021 Jan 19.
Postural tachycardia syndrome (POTS) is a form of autonomic dysfunction characterized by symptoms of orthostatic intolerance, often accompanied by sudomotor dysfunction and gastrointestinal dysmotility. Recently, evidence has accumulated that in a subset of patients, the pathogenesis of dysautonomia may be immune-mediated. The aim of the current report was to evaluate the use of intravenous immunoglobulin (IVIG) treatment in patients with progressive and/or refractory immune-mediated POTS.
We retroactively assessed the effect and tolerance of monthly administered IVIG in six patients using autonomic function testing, standardized symptom questionnaires, and patients' symptom diaries both before and 6 months into IVIG treatment. Objective outcome measures included heart rate increase after 10 min of head-up tilt as well as duration and anhidrotic area in a thermoregulatory sweat test. Subjective outcome measures were patient reports and symptom ratings from the symptom questionnaire.
All patients responded to immunomodulatory treatment, regardless of disease duration. After 6 months of IVIG, symptom severity was reduced by nearly 40%. Autonomic function testing showed improved cardiovascular functioning by 50% and a reduction of anhidrotic areas by one third. Overall, tolerance of IVIG treatment was poor, but could be improved by a reduction in infusion rate, premedication with steroids, and additional intravenous hydration.
Using subjective but also standardized objective measures, the case series describes promising effects of IVIG treatment in POTS patients with immune-mediated dysautonomia. By reducing the infusion rate, pretreatment with steroids, and intravenous hydration, tolerance could be improved, and no patient had to discontinue the treatment.
体位性心动过速综合征（POTS）是一种自主功能障碍，其特征为直立不耐受症状，常伴有自主神经汗腺功能障碍和胃肠道动力障碍。最近，有证据表明，在一部分患者中，自主神经功能障碍的发病机制可能是免疫介导的。本研究旨在评估静脉注射免疫球蛋白（IVIG）治疗进行性和/或难治性免疫介导性 POTS 的效果。
我们回顾性评估了 6 名患者每月接受 IVIG 治疗前后自主功能测试、标准化症状问卷和患者症状日记的效果和耐受性。客观的疗效指标包括头高脚低位 10 分钟后的心率增加，以及体温调节发汗试验中持续时间和无汗面积。主观疗效指标为患者症状问卷的报告和症状评分。
所有患者均对免疫调节治疗有反应，与疾病持续时间无关。接受 IVIG 治疗 6 个月后，症状严重程度降低了近 40%。自主功能测试显示心血管功能改善了 50%，无汗面积减少了三分之一。总体而言，IVIG 治疗的耐受性较差，但通过降低输注速度、类固醇预处理和额外的静脉补液可以改善耐受性，没有患者不得不停止治疗。
使用主观和标准化的客观指标，该病例系列描述了 IVIG 治疗免疫介导性自主神经功能障碍 POTS 患者的有希望的效果。通过降低输注速度、类固醇预处理和静脉补液，可以改善耐受性，没有患者需要停止治疗。
