Hereditary angioedema (HAE) is a rare condition, mostly due to genetic deficiency of complement C1 inhibitor (C1-INH). The rarity of HAE impedes extensive data collection and assessment of the impact of certain factors known to affect the course of this disabling and life-threatening disease. Establishing a global registry could assist to overcome such issues and provides valuable patient data from different countries. The HAE Global Registry is a disease-specific registry, with web-based electronic support, where data are provided by physicians and patients through a dedicated application. We collected data between January 1, 2018, and August 31, 2020. Data on 1297 patients from 29 centers in 5 European countries were collected. At least one attack was recorded for 497 patients during the study period. Overall, 1182 patients were diagnosed with HAE type 1 and 115 with type 2. At the time of database lock, 389 patients were taking long-term prophylactic medication, 217 of which were on danazol. Most recorded attacks affected the abdomen, were generally moderate in severity, and occurred in patients who were not on prophylactic treatment (70.6%, 6244/8848). The median duration of attacks was 780 min (IQR 290–1740) in patients on prophylactic medication and 780 min (IQR 300–1920) in patients not on continuous prophylactic medication. In conclusion, the establishment of a registry for C1-INH-HAE allowed collection of a large amount of data that may help to better understand the clinical characteristics of this disease. This information may enhance patient care and guide future therapeutic decisions.

遗传性血管性水肿 (HAE) 是一种罕见疾病，主要是由于补体 C1 抑制剂 (C1-INH) 的遗传缺陷所致。 HAE 的罕见性阻碍了广泛的数据收集和对已知影响这种致残和危及生命的疾病进程的某些因素的影响的评估。建立全球登记系统可以帮助克服这些问题，并提供来自不同国家的宝贵患者数据。 HAE 全球登记处是一个针对特定疾病的登记处，具有基于网络的电子支持，其中数据由医生和患者通过专用应用程序提供。我们收集了2018年1月1日至2020年8月31日期间的数据。收集了来自5个欧洲国家29个中心的1297名患者的数据。研究期间，497 名患者至少记录了一次发作。总体而言，1182 名患者被诊断为 1 型 HAE，115 名患者被诊断为 2 型 HAE。截至数据库锁定时，389 名患者正在服用长期预防药物，其中 217 名患者正在服用达那唑。大多数记录的发作影响腹部，严重程度一般为中等，发生在未接受预防性治疗的患者中（70.6%，6244/8848）。接受预防性药物治疗的患者中位发作持续时间为 780 分钟（IQR 290-1740），未连续预防性药物治疗的患者为 780 分钟（IQR 300-1920）。总之，C1-INH-HAE 登记的建立可以收集大量数据，可能有助于更好地了解该疾病的临床特征。这些信息可以加强患者护理并指导未来的治疗决策。