Abstract：
Dysautonomia is an abnormal clinical state with multiple etiologies, including autoimmunity. Antiphospholipid antibodies (aPL) are among the autoantibodies that have been associated with autonomic dysfunction. We have observed that an elevated total serum IgM appears to be associated with the presence of aPL in dysautonomia patients. This is a retrospective study analyzing the clinical characteristics of 45 consecutive patients with cardiac autonomic dysfunction and a persistently elevated total serum IgM. 93% of patients were female with a mean age of 32.7 years. Most patients had severely disabling disease, with a mean Karnofsky-like functional ability score of 42% (normal 100%). 93% of patients tested persistently positive for one or more aPL and all patients tested persistently positive for aPL and/or Sjogren\'s antibodies. No patient had lupus specific antibodies. One third of patients experienced one or more thrombotic events and 58% of patients attempting pregnancy experienced pregnancy morbidity. Lastly, 78% of aPL-positive patients treated with antithrombotic therapy experienced 50 to 100% improvement in one or more symptoms (e.g., migraine, cognitive dysfunction) recognized to be responsive to antithrombotic therapy in a subset of aPL-positive patients and 73% of patients treated with and tolerating immune modulatory therapy experienced a positive response. We propose total serum IgM as a reliable and inexpensive test that can be used to identify dysautonomia patients at risk for persistent aPL-positivity. These patients are important to identify as they have a significant risk for thrombosis and pregnancy morbidity and often experience significant symptomatic improvement with antithrombotic therapy and/or immune modulatory therapy.
摘要：
自主神经失调是一种具有多种病因的异常临床状态，包括自身免疫.抗磷脂抗体（aPL）是与自主神经功能障碍相关的自身抗体之一。我们观察到，在自主神经失调患者中，血清总IgM升高似乎与aPL的存在有关。这是一项回顾性研究，分析了45例心脏自主神经功能障碍和总血清IgM持续升高的连续患者的临床特征。93％的患者为女性，平均年龄为32.7岁。大多数患者患有严重致残疾病，平均Karnofsky样功能能力评分为42%(正常100%)。93%的患者检测一种或多种aPL持续阳性，所有患者检测aPL和/或干燥综合征抗体持续阳性。没有患者有狼疮特异性抗体。三分之一的患者经历了一次或多次血栓形成事件，58%的患者尝试妊娠经历了妊娠发病。最后,接受抗血栓治疗的aPL阳性患者中，有78％的一种或多种症状改善了50％至100％（例如，偏头痛,认知功能障碍）在aPL阳性患者亚组中被认为对抗血栓治疗有反应，而接受免疫调节治疗并耐受免疫调节治疗的患者中有73％出现了阳性反应。我们建议将总血清IgM作为一种可靠且廉价的测试，可用于识别有持续aPL阳性风险的自主神经失调患者。这些患者的鉴定是重要的,因为他们具有血栓形成和妊娠发病的显著风险,并且经常经历抗血栓治疗和/或免疫调节治疗的显著症状改善。
