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Abstract：
BACKGROUND: This systematic study aims to assess the global epidemiologic, economic, and humanistic burden of illness associated with all types of hereditary angioedema.
METHODS: A systematic search for articles reporting the epidemiologic, economic, and humanistic burden associated with patients with HAE was conducted using English and Chinese literature databases from the inception to May 23, 2022. The selected studies were assessed for their quality and risk of bias. The study was conducted in accordance with Preferred Reporting Items for Systematic Reviews and Meta-Analyses and registered with the International Prospective Register of Systematic Reviews (PROSPERO; CRD42022352377).
RESULTS: In total, 65 articles that met the search inclusion criteria reported 10,310 patients with HAE, of whom 5861 were female patients. Altogether, 4312 patients (81%) and 479 patients (9%) had type 1 and type 2 HAE, respectively, whereas 422 patients (8%) had HAE-normal C1-INH. The overall prevalence of all types of HAE was between 0.13 and 1.6 cases per 100,000. The mean or median delay from the first onset of a symptom of HAE to confirmed diagnosis ranged from 3.9 to 26 years. The estimated risk of death from asphyxiation was 8.6% for patients with HAE. Hospitalization, medication, unnecessary surgeries, doctor visits, specialist services, and nursing costs are direct expenses that contribute to the growing economic burden. The indirect cost accounted mostly due to missing work ($3402/year) and loss of productivity ($5750/year). Furthermore, impairment of QoL as reported by patient-reported outcomes was observed. QoL measures identified depression, anxiety, and stress to be the most common symptoms for adult patients and children.
CONCLUSIONS: This study highlights the importance of early diagnosis and the need for improving awareness among health care professionals to reduce the burden of HAE on patients and society.
METHODS: A systematic search for articles reporting the epidemiologic, economic, and humanistic burden associated with patients with HAE was conducted using English and Chinese literature databases from the inception to May 23, 2022. The selected studies were assessed for their quality and risk of bias. The study was conducted in accordance with Preferred Reporting Items for Systematic Reviews and Meta-Analyses and registered with the International Prospective Register of Systematic Reviews (PROSPERO; CRD42022352377).
RESULTS: In total, 65 articles that met the search inclusion criteria reported 10,310 patients with HAE, of whom 5861 were female patients. Altogether, 4312 patients (81%) and 479 patients (9%) had type 1 and type 2 HAE, respectively, whereas 422 patients (8%) had HAE-normal C1-INH. The overall prevalence of all types of HAE was between 0.13 and 1.6 cases per 100,000. The mean or median delay from the first onset of a symptom of HAE to confirmed diagnosis ranged from 3.9 to 26 years. The estimated risk of death from asphyxiation was 8.6% for patients with HAE. Hospitalization, medication, unnecessary surgeries, doctor visits, specialist services, and nursing costs are direct expenses that contribute to the growing economic burden. The indirect cost accounted mostly due to missing work ($3402/year) and loss of productivity ($5750/year). Furthermore, impairment of QoL as reported by patient-reported outcomes was observed. QoL measures identified depression, anxiety, and stress to be the most common symptoms for adult patients and children.
CONCLUSIONS: This study highlights the importance of early diagnosis and the need for improving awareness among health care professionals to reduce the burden of HAE on patients and society.
摘要：
背景：这项系统研究旨在评估全球流行病学，经济,与所有类型的遗传性血管性水肿相关的人文疾病负担。
方法：系统搜索报告流行病学的文章，经济,从开始至2022年5月23日，使用英文和中文文献数据库对HAE患者的人文负担进行了研究。对选定的研究进行质量和偏倚风险评估。该研究是根据系统评价和荟萃分析的首选报告项目进行的，并在国际系统评价前瞻性注册中心（PROSPERO;CRD42022352377）注册。
结果：总计，符合检索纳入标准的65篇文章报道了10310例HAE患者，其中5861人为女性患者。总之,4312例患者（81%）和479例患者（9%）患有1型和2型HAE，分别,而422例患者（8%）有HAE-正常C1-INH。所有类型的HAE的总体患病率为每100,000例0.13至1.6例。从HAE症状的首次发作到确诊的平均或中值延迟范围为3.9至26年。HAE患者窒息死亡的估计风险为8.6%。住院治疗,药物,不必要的手术,看医生,专业服务,护理费用是导致经济负担不断增加的直接费用。间接成本主要是由于缺少工作（3402美元/年）和生产力损失（5750美元/年）。此外，观察到患者报告结局报告的QoL受损.QoL措施确定抑郁症，焦虑,压力是成人患者和儿童最常见的症状。
结论：本研究强调了早期诊断的重要性以及提高卫生保健专业人员意识以减轻HAE对患者和社会的负担的必要性。
方法：系统搜索报告流行病学的文章，经济,从开始至2022年5月23日，使用英文和中文文献数据库对HAE患者的人文负担进行了研究。对选定的研究进行质量和偏倚风险评估。该研究是根据系统评价和荟萃分析的首选报告项目进行的，并在国际系统评价前瞻性注册中心（PROSPERO;CRD42022352377）注册。
结果：总计，符合检索纳入标准的65篇文章报道了10310例HAE患者，其中5861人为女性患者。总之,4312例患者（81%）和479例患者（9%）患有1型和2型HAE，分别,而422例患者（8%）有HAE-正常C1-INH。所有类型的HAE的总体患病率为每100,000例0.13至1.6例。从HAE症状的首次发作到确诊的平均或中值延迟范围为3.9至26年。HAE患者窒息死亡的估计风险为8.6%。住院治疗,药物,不必要的手术,看医生,专业服务,护理费用是导致经济负担不断增加的直接费用。间接成本主要是由于缺少工作（3402美元/年）和生产力损失（5750美元/年）。此外，观察到患者报告结局报告的QoL受损.QoL措施确定抑郁症，焦虑,压力是成人患者和儿童最常见的症状。
结论：本研究强调了早期诊断的重要性以及提高卫生保健专业人员意识以减轻HAE对患者和社会的负担的必要性。
