PDF(Pubmed)
Abstract：
Hereditary Angioedema (HAE) is a rare and disabling disease for which early diagnosis and effective therapy are critical. This revision and update of the global WAO/EAACI guideline on the diagnosis and management of HAE provides up-to-date guidance for the management of HAE. For this update and revision of the guideline, an international panel of experts reviewed the existing evidence, developed 28 recommendations, and established consensus by an online DELPHI process. The goal of these recommendations and guideline is to help physicians and their patients in making rational decisions in the management of HAE with deficient C1-inhibitor (type 1) and HAE with dysfunctional C1-inhibitor (type 2), by providing guidance on common and important clinical issues, such as: 1) How should HAE be diagnosed? 2) When should HAE patients receive prophylactic on top of on-demand treatment and what treatments should be used? 3) What are the goals of treatment? 4) Should HAE management be different for special HAE patient groups such as children or pregnant/breast feeding women? 5) How should HAE patients monitor their disease activity, impact, and control? It is also the intention of this guideline to help establish global standards for the management of HAE and to encourage and facilitate the use of recommended diagnostics and therapies for all patients.
摘要：
遗传性血管性水肿（HAE）是一种罕见且致残的疾病，其早期诊断和有效治疗至关重要。WAO/EAACI关于HAE诊断和管理的全球指南的修订和更新为HAE的管理提供了最新的指导。对于本指南的更新和修订，一个国际专家小组审查了现有的证据，提出了28项建议，并通过在线DELPHI流程建立共识。这些建议和指南的目的是帮助医生及其患者在C1抑制剂缺陷型HAE（1型）和C1抑制剂功能失调型HAE（2型）的管理中做出合理的决定。通过对常见和重要的临床问题提供指导，如:1)HAE应如何诊断?影响，本指南还旨在帮助建立HAE管理的全球标准，并鼓励和促进对所有患者使用推荐的诊断和治疗方法。
