Department of Medicine, Denver University of Colorado Anschutz Medical Campus, Aurora, CO.
Department of Neurology, Eastern Virginia Medical School, Norfolk, VA.
Am J Ther. 2019 Sep/Oct;26(5):570-582. doi: 10.1097/MJT.0000000000000778.
Intravenous immunoglobulin (IVIG) has recognized efficacy in autoimmune peripheral nerve disorders, but there has been limited study of the use of IVIG in autoimmune dysautonomias.
To determine the efficacy and safety of IVIG in patients with disabling, refractory autoimmune dysautonomias, including patients with postural tachycardia syndrome and gastrointestinal dysmotility.
Patients with one or more autonomic disorder(s) and persistent serological evidence for autoimmunity who were unable to work or attend school despite usual treatments for dysautonomia were treated with IVIG for at least 3 months at a dose of at least 1 gm/kg monthly.
Outcome measures included the composite autonomic symptom scale 31 survey and a functional ability score.
There were 38 patients, 84% female and mean age of 28.4 years. Of patients, 83.5% improved on IVIG as defined by at least 20% improvement in the composite autonomic symptom scale 31 and/or functional ability score. The mean pretreatment functional ability score was 21% (mostly bedridden), which improved to a mean of 74% (nearing able to return to work/school) for responsive patients after at least 1 year of IVIG. The mean time to the first sign of response was 5.3 weeks. There were no serious adverse events. The Mayo autoimmune dysautonomia panel antibodies and traditional Sjögren antibodies were present in only 13% and 8% of patients, respectively, but antiphospholipid antibodies and novel Sjögren antibodies were present in 76% and 42% of patients, respectively.
There is increasing evidence that IVIG is safe and effective in a subset of patients with autonomic disorders and evidence for autoimmunity. A 4-month IVIG trial should be considered in severely affected patients who are refractory to lifestyle and pharmacological therapies. Antiphospholipid antibodies and novel Sjögren antibodies are often present in these patients and correlate with a high response rate to IVIG.
静脉注射免疫球蛋白（IVIG）已被证实对自身免疫性周围神经疾病有效，但在自身免疫性自主神经功能障碍中使用 IVIG 的研究有限。
确定 IVIG 在患有致残性、难治性自身免疫性自主神经功能障碍的患者中的疗效和安全性，包括体位性心动过速综合征和胃肠动力障碍患者。
患有一种或多种自主神经障碍且持续存在自身免疫血清学证据的患者，尽管接受了常规的自主神经功能障碍治疗，但仍无法工作或上学，这些患者接受了至少 3 个月的 IVIG 治疗，剂量为至少 1 克/公斤/月。
结果测量包括综合自主症状量表 31 调查和功能能力评分。
共有 38 例患者，84%为女性，平均年龄为 28.4 岁。根据综合自主症状量表 31 和/或功能能力评分至少改善 20%，83.5%的患者对 IVIG 有反应。治疗前功能能力评分的平均值为 21%（主要卧床不起），对有反应的患者，经过至少 1 年的 IVIG 治疗后，平均提高到 74%（接近能够重返工作/学校）。首次出现反应的平均时间为 5.3 周。没有严重的不良事件。梅奥自身免疫性自主神经功能障碍小组抗体和传统干燥综合征抗体分别仅在 13%和 8%的患者中存在，而抗磷脂抗体和新型干燥综合征抗体分别在 76%和 42%的患者中存在。
越来越多的证据表明，IVIG 在一组自主神经障碍和自身免疫证据的患者中是安全有效的。对于对生活方式和药物治疗有抵抗的严重受影响患者，应考虑进行为期 4 个月的 IVIG 试验。抗磷脂抗体和新型干燥综合征抗体在这些患者中经常存在，并与 IVIG 的高反应率相关。
