Chong-Neto Herberto José
Division of Allergy and Immunology, Complexo Hospital de Clínicas, Federal University of Paraná, Curitiba, Paraná, Brazil.
World Allergy Organ J. 2023 Mar 20;16(3):100758. doi: 10.1016/j.waojou.2023.100758. eCollection 2023 Mar.
Hereditary angioedema (HAE) is a rare disorder that causes unpredictable and debilitating cutaneous and submucosal edema and can lead to death. HAE can impair patients' ability to perform daily activities, proportional to pain severity, with patients reporting lower productivity, missed time from work or school and potentially resulting in missed career and educational opportunities. Many patients with HAE experience a significant psychological burden, including anxiety and depression. Available treatment aims to prevent and/or treat HAE attacks as they occur, to reduce morbidity and mortality and, finally, to improve health-related quality of life. Two different validated specific angioedema instruments are available to assess patients' quality of life. The Angioedema Quality of Life Questionnaire (AE-QoL) examines diagnosed patients' quality of life but is not specific for HAE. The disease-specific questionnaire is the Hereditary Angioedema Quality of Life (HAE-QoL), and the first used for hereditary angioedema with C1 inhibitors (C1-INH) deficiency. These quality-of-life instruments are helpful to the HAE patients' assessment and to the development of better therapeutic strategies as clinical tools, as defined by international guidelines. Considering this context, this review was conducted to compare the effects of acute vs. long-term prophylaxis on HAE patients' health-related quality of life. In addition, the prevalence of anxiety and depression among these individuals was also reviewed.
遗传性血管性水肿(HAE)是一种罕见的疾病,可导致不可预测且使人衰弱的皮肤和黏膜下水肿,并可能导致死亡。HAE会损害患者进行日常活动的能力,其程度与疼痛严重程度成正比,患者报告工作效率降低、误工或误学,甚至可能错失职业和教育机会。许多HAE患者承受着巨大的心理负担,包括焦虑和抑郁。现有的治疗旨在预防和/或治疗HAE发作,降低发病率和死亡率,并最终改善与健康相关的生活质量。有两种经过验证的不同的特定血管性水肿评估工具可用于评估患者的生活质量。血管性水肿生活质量问卷(AE-QoL)用于检查已确诊患者的生活质量,但并非HAE所特有。针对该疾病的问卷是遗传性血管性水肿生活质量问卷(HAE-QoL),它首次用于伴有C1抑制剂(C1-INH)缺乏的遗传性血管性水肿。这些生活质量评估工具作为国际指南所定义的临床工具,有助于对HAE患者进行评估,并有助于制定更好的治疗策略。考虑到这一背景,本综述旨在比较急性预防与长期预防对HAE患者与健康相关生活质量的影响。此外,还对这些患者中焦虑和抑郁的患病率进行了综述。