Abstract：
Hereditary angioedema (HAE) is a rare genetic disease with significant morbidity and mortality for which early diagnosis and effective therapy are critical. Many Asia Pacific (AP) countries still lack access to diagnostic tests and evidence-based therapies. Epidemiologic data from the AP is needed to formulate regional guidelines to improve standards of care for HAE.
To investigate the estimated minimal prevalence, needs, and potential interventions for the diagnosis and management of HAE in the AP.
A structured questionnaire was distributed to representative experts from member societies of the Asia Pacific Association of Allergy, Asthma and Clinical Immunology. Patient profiles and the presence of diagnostic facilities or tests, regional and national HAE guidelines, and patient support groups were reported and compared.
Completed questionnaires were received from 14 representatives of 12 member countries and territories, representing 46% of the world population. Overall minimal prevalence of HAE in the AP region was 0.02/100,000 population, with significant heterogeneity across different centers. Only one-half and one-third had registered on-demand and prophylactic medications, respectively. Few had patient support groups (58%) or regional guidelines (33%), and their existence was associated with the availability of HAE-specific medications. Availability of C1-inhibitor level testing was associated with a lower age at HAE diagnosis (P = .017).
Hereditary angioedema in the AP differs from that in Western countries. Hereditary angioedema-specific medications were registered in only a minority of countries and territories, but those with patient support groups or regional guidelines were more likely to have better access. Asia Pacific-specific consensus and guidelines are lacking and urgently needed.
To investigate the estimated minimal prevalence, needs, and potential interventions for the diagnosis and management of HAE in the AP.
A structured questionnaire was distributed to representative experts from member societies of the Asia Pacific Association of Allergy, Asthma and Clinical Immunology. Patient profiles and the presence of diagnostic facilities or tests, regional and national HAE guidelines, and patient support groups were reported and compared.
Completed questionnaires were received from 14 representatives of 12 member countries and territories, representing 46% of the world population. Overall minimal prevalence of HAE in the AP region was 0.02/100,000 population, with significant heterogeneity across different centers. Only one-half and one-third had registered on-demand and prophylactic medications, respectively. Few had patient support groups (58%) or regional guidelines (33%), and their existence was associated with the availability of HAE-specific medications. Availability of C1-inhibitor level testing was associated with a lower age at HAE diagnosis (P = .017).
Hereditary angioedema in the AP differs from that in Western countries. Hereditary angioedema-specific medications were registered in only a minority of countries and territories, but those with patient support groups or regional guidelines were more likely to have better access. Asia Pacific-specific consensus and guidelines are lacking and urgently needed.
摘要：
背景：遗传性血管性水肿（HAE）是一种罕见的遗传性疾病，具有严重的发病率和死亡率，早期诊断和有效治疗至关重要。许多亚太（AP）国家仍然缺乏诊断测试和循证疗法。需要来自AP的流行病学数据来制定区域指南，以提高HAE的护理标准。
目的：调查估计的最低患病率，AP中HAE诊断和管理的需求和潜在干预措施。
方法：向亚太变态反应协会成员协会的代表性专家分发了一份结构化问卷，哮喘和临床免疫学（APAAACI）。患者资料和诊断设施/测试的存在，报告并比较了地区/国家HAE指南和患者支持组.
结果：收到了来自12个成员国/地区的14名代表的完整问卷，占世界人口的46%。AP地区HAE的总体最低患病率为每100,000人口0.02，在不同中心具有显著的异质性。只有一半和三分之一的人登记了按需和预防性药物，分别。很少有患者支持小组（58％）或区域指南（33％），它们的存在与HAE特异性药物的可用性相关。C1抑制剂水平检测的可用性与HAE诊断年龄较低相关(p=0.017)。
结论：AP中的HAE不同于西方国家。HAE特定药物仅在少数国家/地区注册，但那些有患者支持小组或地区指南的人更有可能获得更好的访问权限.缺乏和迫切需要AP特定的共识和准则。
目的：调查估计的最低患病率，AP中HAE诊断和管理的需求和潜在干预措施。
方法：向亚太变态反应协会成员协会的代表性专家分发了一份结构化问卷，哮喘和临床免疫学（APAAACI）。患者资料和诊断设施/测试的存在，报告并比较了地区/国家HAE指南和患者支持组.
结果：收到了来自12个成员国/地区的14名代表的完整问卷，占世界人口的46%。AP地区HAE的总体最低患病率为每100,000人口0.02，在不同中心具有显著的异质性。只有一半和三分之一的人登记了按需和预防性药物，分别。很少有患者支持小组（58％）或区域指南（33％），它们的存在与HAE特异性药物的可用性相关。C1抑制剂水平检测的可用性与HAE诊断年龄较低相关(p=0.017)。
结论：AP中的HAE不同于西方国家。HAE特定药物仅在少数国家/地区注册，但那些有患者支持小组或地区指南的人更有可能获得更好的访问权限.缺乏和迫切需要AP特定的共识和准则。
