Department of Medicine, Division of Clinical Immunology and Allergy, University of Toronto, Toronto, Ontario, Canada.
Division of Allergy and Immunology, Department of Medicine, University of British Columbia, Vancouver, British Columbia, Canada.
Ann Allergy Asthma Immunol. 2018 Nov;121(5):598-603. doi: 10.1016/j.anai.2018.06.017. Epub 2018 Jun 26.
Hereditary angioedema (HAE) is a rare disease that has significant morbidity and may be potentially fatal because of airway obstruction.
To determine practice patterns in physicians treating HAE.
A survey was designed to determine HAE practice patterns among Canadian physicians. These physicians were identified by sending the survey to members of 3 physician organizations (Canadian Hereditary Angioedema Network, Canadian Society of Clinical Immunology and Allergy, and Canadian Hematology Society).
Thirty-six physicians responded to the survey. Thirty-four physicians were included in the analysis. Most referrals to HAE-treating physicians were from family and emergency department physicians. The most common sites of swelling reported by patients to physicians were facial, peripheral, and abdominal. A mean of 53.9% of patients with type 1 and 2 HAE and 53.4% of patients with HAE with normal C1 esterase inhibitor were undergoing long-term prophylaxis. A mean of 41.9%, 19.4%, and 93.5% of respondents had some patients taking danazol, tranexamic acid, and C1-esterase inhibitor, respectively. Most physicians believed that severity and frequency of attacks were the most important determinants in deciding when to use prophylaxis. A mean of 88.2% of physicians used C1-esterase inhibitor to treat acute attacks and 79.4% used icatibant. All respondents were aware of HAE guidelines.
Physicians are using guidelines to support their practice and using agents suggested by guidelines with confidence. C1 inhibitor is being used widely for prophylaxis and treatment of acute attacks along with icatibant. However, certain special patient populations may require additional focus in future guidelines.
遗传性血管性水肿（HAE）是一种罕见疾病，发病率高，可能因气道阻塞而导致潜在致命。
确定治疗 HAE 的医生的实践模式。
设计了一项调查，以确定加拿大医生治疗 HAE 的实践模式。通过向 3 个医师组织（加拿大遗传性血管性水肿网络、加拿大临床免疫学和过敏学会以及加拿大血液学学会）的成员发送调查，确定了这些医师。
有 36 名医生对调查做出了回应。34 名医生被纳入分析。向 HAE 治疗医生的大多数转介来自家庭医生和急诊医生。患者向医生报告的最常见肿胀部位是面部、外周和腹部。1 型和 2 型 HAE 患者中，有 53.9%的患者和 C1 酯酶抑制剂正常的 HAE 患者中，有 53.4%的患者接受长期预防治疗。有 41.9%、19.4%和 93.5%的受访者分别有一些患者服用达那唑、氨甲环酸和 C1 酯酶抑制剂。大多数医生认为，发作的严重程度和频率是决定何时使用预防治疗的最重要决定因素。有 88.2%的医生平均使用 C1 酯酶抑制剂治疗急性发作，79.4%的医生使用艾替班特。所有受访者都了解 HAE 指南。
医生正在使用指南来支持他们的实践，并自信地使用指南推荐的药物。C1 抑制剂广泛用于预防和治疗急性发作，同时还使用艾替班特。然而，某些特殊患者群体可能需要在未来的指南中给予更多关注。
