Keith Paul K, Lacuesta Gina, Goodyear Dawn, Betschel Stephen D, Yap Belinda, Dansereau Marie-France, Tanios Nataly, El-Sayegh Rami, Machnouk Maye, Mahfouz Hachem, Martin Adriana, Waserman Susan
Department of Medicine, McMaster University, 3V47 HSC 1280 Main St W, Hamilton, ON, L8S 4K1, Canada.
Dalhousie University, Halifax, NS, Canada.
Allergy Asthma Clin Immunol. 2025 Mar 19;21(1):13. doi: 10.1186/s13223-025-00953-8.
Evidence linking hereditary angioedema (HAE) to the potential association of developing other comorbidities, and how it is affected by HAE treatment is needed. The objective of this study is to identify comorbidities and measure the prevalence in HAE patients, compared to the prevalence in the general population using multiple Canadian sources when available.
A quantitative survey design via a self-administered anonymous online questionnaire was conducted from October 13, 2022, to January 11, 2023. Respondents were individuals with HAE, enrolled in the CSL Behring patient support program (CSL Behring PLUS+; PSP).
This study included 123 patients (81% female; 60% HAE-1/HAE-2, 24% HAE Normal C1-INH (nC1-INH), 16% unsure of HAE type; 85% of patients were on long-term prophylaxis plus on-demand). Patients reported using the following HAE treatments: C1-esterase inhibitor (subcutaneous or intravenous), lanadelumab, icatibant, danazol, and tranexamic acid. Respondents (69%) reported at least one: autoimmune condition, asthma, or allergy. Reported autoimmune conditions (psoriasis, rheumatoid arthritis, inflammatory bowel disease, chronic urticaria, lupus, and psoriatic arthritis) were much higher than the general population (31% versus 5-8%). Patient-reported allergies were two times higher than the general population (54% versus 27%; i.e., aeroallergens) and asthma rates nearly two times higher than the general population (17% versus 8-11%).
This cohort of HAE patients, most of whom were on prophylaxis, reported an increased prevalence of certain comorbidities compared to the general Canadian population. Healthcare professionals should be aware of the potentially increased risk of autoimmune conditions, allergies, and asthma in patients with HAE.
需要有证据证明遗传性血管性水肿（HAE）与其他合并症发生的潜在关联，以及HAE治疗如何对其产生影响。本研究的目的是识别HAE患者的合并症，并与使用加拿大多个可用数据源的普通人群患病率进行比较，以测量HAE患者的患病率。
于2022年10月13日至2023年1月11日通过自我管理的匿名在线问卷进行定量调查设计。受访者为参加赛诺菲（CSL Behring）患者支持计划（CSL Behring PLUS+；PSP）的HAE患者。
本研究纳入了123例患者（81%为女性；60%为HAE-1/HAE-2型，24%为HAE正常C1-抑制剂（nC1-INH）型，16%不确定HAE类型；85%的患者接受长期预防治疗并按需治疗）。患者报告使用以下HAE治疗方法：C1-酯酶抑制剂（皮下或静脉注射）、拉那度单抗、依卡替班、达那唑和氨甲环酸。69%的受访者报告至少有一种：自身免疫性疾病、哮喘或过敏。报告的自身免疫性疾病（银屑病、类风湿性关节炎、炎症性肠病、慢性荨麻疹、狼疮和银屑病关节炎）远高于普通人群（31%对5-8%）。患者报告的过敏率是普通人群的两倍（54%对27%；即吸入性过敏原），哮喘发生率几乎是普通人群的两倍（17%对8-11%）。
与加拿大普通人群相比，这组大多数接受预防治疗的HAE患者报告某些合并症的患病率有所增加。医疗保健专业人员应意识到HAE患者自身免疫性疾病、过敏和哮喘的潜在风险增加。
