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伯格斯病（血栓闭塞性脉管炎）的免疫吸附：一种有前途的治疗选择：临床常规护理中连续患者队列的结果

Peter Klein-Weigel、Theresa Sophia Volz、Beate Gutsche-Petrak、Joana M. Boehnlein、Anne Bohlen、Siegrid Dreusicke、Jana Valerius、Marion Bimmler、Petra Hempel、Leonora Zange、Sebastian Schopp 和 Saban Elitok

背景：布尔格氏病（TAO，血栓闭塞性脉管炎）是一种影响中小动脉和静脉的炎性血管疾病，导致肢端或危及肢体的缺血综合征和/或血栓性静脉炎，截肢率很高。免疫组织病理学和血清学数据揭示了 TAO 免疫发病机制的新范式。基于这一假设，免疫吸附 (IA) 被成功引入治疗范围，TAO 中存在 G 蛋白偶联自身抗体，并被 IA 成功消除。

目的：我们更新了观察性队列研究的结果，其中包括在临床常规护理中连续接受 IA 治疗的 TAO 患者。

患者和方法： 2012年12月至2016年2月，22名TAO患者在我院接受IA治疗。回顾性地，必须排除 3 名患者（发现 Lp(a)- 浓度升高、存在动脉粥样硬化性冠状动脉病变、血样丢失），留下 19 名患者进行最终分析（17 名男性，2 名女性；平均年龄 40 岁（20- 54）年）。IA 在为期五天的课程中使用 Fesenius-GlobaffinR 吸附剂进行，预期清除 2.5 倍的血浆体积。使用特定的市售 ELISA 技术对 G 蛋白偶联 -AAB 进行分析。临床随访包括定期门诊和/或电话联系居住在较偏远地区的患者。数据通过描述性统计呈现。

结果： G-protein receptor autoantibodies (AAB) were present in 14 of our patients (74%), with 1 AAB in 5 and multiple AAB in 9 patients. The presence of a clustering of AAB directed against the α1-receptor and endothelin Areceptor was seen in 9 out of the 14 AAB-positive patients (64%). AAB directed against ET-A-receptors never appeared without AAB directed against the α1-receptor and were exclusively directed against the extracellular receptor-loop 1. Immediately after a five day course of IA, 12 out of 14 AAB-positive patients were free of AAB (85%). Follow-up data was available in 15 patients. During a mean follow-up period of 3 month (0-35 month) there were no disease flairs. In all but one patient skin lesions healed. Pain scale values decreased from 7.0 (5-9) to 2.0 (0-5). Minor amputations already anticipated before IA were performed in two patients without complications. Only one patient underwent major-amputation after a failed surgical bypass procedure of doubtful indication with prior subcritical forefoot-tcpO2-values during follow-up in our institution. There was a high rate of smoking cessations (13 active smokers before IA, 3 during last follow-up) due to a close monitoring and admonition.

结论：虽然 G 蛋白偶联受体 -AAB 在 TAO 中的确切作用尚未确定，但我们能够重现我们之前发表的这些 AAB 聚类结果，以及在这个更大的队列中通过 IA 成功消除它们的结果，这预计有益的临床过程。IA 或许能够稳定病程，但戒烟率异常高，因此无法准确定义其对临床结果的确切贡献。