Division of Rheumatology, Allergy, and Immunology, Department of Medicine, Mount Sinai School of Medicine, New York, NY.
Division of Rheumatology, Allergy, and Immunology, Department of Medicine, University of California San Diego, La Jolla, Calif.
J Allergy Clin Immunol Pract. 2021 Jan;9(1):132-150.e3. doi: 10.1016/j.jaip.2020.08.046. Epub 2020 Sep 6.
Scientific and clinical progress together with the development of effective novel therapeutic options has engendered multiple important changes in the diagnosis and management of hereditary angioedema (HAE). We now update and extend the 2013 United States Hereditary Angioedema Association Medical Advisory Board guidelines for the treatment and management of HAE. The guidelines are based on a comprehensive literature review with recommendations indicating both the strength of our recommendation and the quality of the underlying evidence. Guidelines are provided regarding the classification, diagnosis, on-demand treatment, prophylactic treatment, special considerations for women and children, development of a comprehensive management and monitoring plan, and assessment of burden of illness for both HAE due to C1 inhibitor deficiency and HAE with normal C1 inhibitor. Advances in HAE treatment now allow the development of management plans that can help many patients with HAE lead a normal life. Achieving this goal requires that physicians be familiar with the diagnostic and therapeutic transformations that have occurred in recent years.
科学和临床的进步，以及有效新型治疗选择的发展，使得遗传性血管性水肿（HAE）的诊断和管理发生了多项重要变化。我们现在更新和扩展了 2013 年美国遗传性血管性水肿协会医学顾问委员会关于 HAE 治疗和管理的指南。这些指南是基于全面的文献回顾，建议表明了我们建议的力度和基础证据的质量。提供了有关分类、诊断、按需治疗、预防治疗、女性和儿童的特殊考虑因素、制定全面管理和监测计划以及评估 C1 抑制剂缺乏和正常 C1 抑制剂的 HAE 患者的疾病负担的指南。HAE 治疗的进展现在可以制定管理计划，帮助许多 HAE 患者过上正常的生活。要实现这一目标，医生必须熟悉近年来发生的诊断和治疗转变。
