Supplementary Material for: THE RELATIONSHIP BETWEEN SURGICAL PROCEDURES AND ANGIOEDEMA ATTACKS IN HEREDITARY ANGIOEDEMA

Introduction: Surgical interventions can trigger angioedema attacks in hereditary angioedema (HAE). The aim of this study was to assess the incidence of perioperative angioedema and identify associated risk factors.
Methods: This retrospective study included HAE patients diagnosed between 1999 and 2024 at a tertiary adult allergy clinic. Data on surgical procedures and perioperative angioedema were analyzed.
Results: Of 102 HAE patients, 28 were excluded due to incomplete data, leaving 74 patients (46 female, 62.2%). Fifty-three patients underwent 94 surgeries, with the most common being gynecological (27, 28.7%), abdominal (27, 28.7%), and otorhinolaryngological (16, 17.0%). Of 54 surgeries before HAE diagnosis, 23 (42.5%) were abdominal. Among 27 abdominal surgeries, 17 (62.9%) occurred in patients with gastrointestinal angioedema prior to diagnosis. Of 40 surgeries after diagnosis, 31 (77.5%) received preoperative short term prophylaxis (STP), mostly plasma-derived C1 esterase inhibitor concentrate (27, 87.1%). Perioperative angioedema occurred in 28 (29.8%) surgeries, with a median recovery of 48 hours. In surgeries after diagnosis, attacks occurred in 7 out of 31 surgeries (22.6%) with STP and 2 out of 9 (22.2%) without. Among gynecological surgeries, 22 cesarean sections were performed. No significant difference in attack frequency was found between cesarean sections with STP (3, 27.3%) and without (2, 18.2%, p=0.611).
Conclusion: Our study found that abdominal attacks before HAE diagnosis are consistent with literature, underscoring their importance for early diagnosis. The angioedema rate was similar with or without STP before cesarean section, suggesting STP may not be necessary. Further research is needed to optimize HAE management in surgery.

引言：手术干预可诱发遗传性血管性水肿（hereditary angioedema, HAE）患者的血管性水肿发作。本研究旨在评估围手术期血管性水肿（perioperative angioedema）的发生率并明确相关危险因素。
方法：本回顾性研究纳入了1999年至2024年间于某三级成人过敏专科门诊确诊的遗传性血管性水肿患者，对手术操作及围手术期血管性水肿相关数据进行分析。
结果：共纳入102例遗传性血管性水肿患者，其中28例因数据不全被排除，最终纳入74例患者（女性46例，占比62.2%）。53例患者共接受94次手术，其中最常见的手术类型为妇科手术（27次，占比28.7%）、腹部手术（27次，占比28.7%）及耳鼻咽喉科手术（16次，占比17.0%）。在确诊遗传性血管性水肿前完成的54次手术中，23次（42.5%）为腹部手术。在27次腹部手术中，17次（62.9%）的手术对象为确诊前已出现胃肠道血管性水肿的患者。确诊后完成的40次手术中，31次（77.5%）接受了术前短期预防（short term prophylaxis, STP）治疗，其中多数采用血浆源性C1酯酶抑制剂浓缩物（plasma-derived C1 esterase inhibitor concentrate）（27次，占比87.1%）。围手术期血管性水肿发作见于28次手术（占比29.8%），患者中位恢复时长为48小时。在确诊后接受的手术中，接受术前短期预防的31次手术里有7次（22.6%）发作血管性水肿，未接受预防的9次手术中有2次（22.2%）发作。妇科手术中共完成22例剖宫产术。接受术前短期预防的剖宫产术（3例，占比27.3%）与未接受预防的剖宫产术（2例，占比18.2%）的血管性水肿发作频率无显著差异（p=0.611）。
结论：本研究发现，遗传性血管性水肿确诊前的腹部血管性水肿发作情况与现有文献报道一致，凸显了其对早期诊断的重要意义。剖宫产术前是否接受短期预防的血管性水肿发作率无明显差异，提示术前短期预防或并非必需。未来仍需开展进一步研究以优化遗传性血管性水肿患者的围手术期管理方案。