Complex regional pain syndrome and dysautonomia in a 14-year-old girl responsive to therapeutic plasma exchange
Complex regional pain syndrome and dysautonomia in a 14-year-old girl responsive to therapeutic plasma exchange
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DOI：
10.1002/jca.21407
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发表时间：
2016-08-01
影响因子：
1.5
通讯作者：
Tormey, Christopher A.
中科院分区：
文献类型：
--
作者：
Hendrickson, Jeanne E.;Hendrickson, Emma T.;Tormey, Christopher A.
反射性交感神经营养不良，也称为复杂性区域疼痛综合征（CRPS），最近已被证明与抗β 2-肾上腺素能和毒蕈碱M2受体的自身抗体有关。除了疼痛和催汗/血管痉挛症状外，还在CRPS患者亚组中观察到自主神经功能障碍。尽管其严重性，但迄今为止描述的CRPS的有效疗法很少。我们报告了一例14岁的女孩，她的右腿CRPS和自主神经功能障碍（胃轻瘫，体位性心动过速）难治性多种治疗，成功地治疗了治疗性血浆置换（TPE）与白蛋白替代。该患者除了烟碱乙酰胆碱受体神经节自身抗体外，还具有血清抗2-肾上腺素能和毒蕈碱M2受体自身抗体，在2周内接受了5次TPE的初始疗程。她对TPE表现出临床反应，表现为疲劳和胃轻瘫迅速改善，腿部疼痛和出汗/血管舒缩发作逐渐但显着改善。负荷程序后，患者接受利妥昔单抗治疗。她继续需要定期TPE以维持缓解，并考虑长期额外的免疫抑制。尽管还需要进一步研究，TPE（联合免疫抑制）可能是一种适用于可检测到自身抗体的CRPS患者的治疗方法，因为它适用于具有针对神经元表面受体的自身抗体的疾病，如重症肌无力或Lambert Eaton肌无力综合征。临床单采31：368-374，2016年。(c)2015 Wiley Periodicals，Inc.
Reflex sympathetic dystrophy, also known as complex regional pain syndrome (CRPS), has recently been shown to be associated with autoantibodies against 2-adrenergic and muscarinic M2 receptors. In addition to pain and sudomotor/vasomotor symptoms, dysautonomia is also observed in a subset of CRPS patients. Despite its severity, there are few effective therapies for CRPS described to date. We report a case of a 14-year-old girl with CRPS of her right leg and dysautonomia (gastroparesis, postural tachycardia) refractory to multiple therapies, successfully treated with therapeutic plasma exchange (TPE) with albumin replacement. The patient, who has serum anti 2-adrenergic and muscarinic M2 receptor autoantibodies in addition to nicotinic acetylcholine receptor ganglionic autoantibodies, underwent an initial course of five TPEs over a 2-week period. She demonstrated a clinical response to TPE as manifested by a rapid improvement in her fatigue and gastroparesis, with a gradual yet significant improvement in her leg pain and sudomotor/vasomotor flares. Following the loading procedures, the patient was treated with rituximab. She continues to require periodic TPE to maintain a remission, with additional immunosuppression being considered long term. Although further studies are needed, TPE (in combination with immunosuppression) may be an appropriate therapy for CRPS patients with detectable autoantibodies, as it is for better characterized diseases with autoantibodies against neuronal surface receptors such as myasthenia gravis or Lambert Eaton myasthenic syndrome. J. Clin. Apheresis 31:368-374, 2016. (c) 2015 Wiley Periodicals, Inc.