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：外科手术可诱发遗传性血管性水肿（hereditary angioedema, HAE）患者出现血管性水肿发作。本研究旨在评估围手术期血管性水肿的发生率，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，在某三级成人过敏专科诊所确诊的HAE患者。对手术操作及围手术期血管性水肿相关数据进行了分析。
结果：本研究初始纳入102例HAE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HAE确诊前实施的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。在HAE确诊后实施的40次手术中，31次（77.5%）接受了术前短期预防（short term prophylaxis, STP）治疗，其中绝大多数使用血浆源性C1酯酶抑制剂浓缩剂（27次，占比87.1%）。28次（29.8%）手术出现了围手术期血管性水肿发作，患者中位恢复时间为48小时。在确诊后实施的手术中，接受STP治疗的31次手术中有7次（22.6%）出现发作，未接受STP治疗的9次手术中有2次（22.2%）出现发作。妇科手术中，共完成22例剖宫产手术。接受STP治疗的剖宫产手术（3次，27.3%）与未接受STP治疗的剖宫产手术（2次，18.2%）的发作频率无显著差异（p=0.611）。
结论：本研究发现，HAE确诊前出现腹部血管性水肿发作的情况与现有文献报道一致，凸显了该症状对早期诊断的重要价值。剖宫产术前无论是否使用STP，血管性水肿的发生率均相近，提示STP或非必需。未来仍需开展进一步研究，以优化HAE患者的围手术期管理方案。