Uncovering the true burden of hereditary angioedema due to C1-inhibitor deficiency: A focus on the Asia-Pacific region

Abstract

Hereditary angioedema (HAE) due to C1-inhibitor deficiency or dysfunction is a rare genetic disorder that causes recurrent episodes of swelling in various parts of the body. Treatment goals of HAE aim to “normalize” life for all patients; however, lack of diagnostic facilities and limited access to effective treatment options in developing nations cause delays in diagnosis and place a significant burden on patients. In this review, we aim to highlight the burden of disease caused by C1-inhibitor HAE across the Asia-Pacific region, considering its epidemiology, morbidity and mortality, and socioeconomic and psychological impact. We also review the availability of guideline-recommended diagnostic facilities and treatments, and how patients are currently managed. Data were collected from published literature and HAE experts in the region, who provided information regarding diagnosis and management in their countries. Current practice was reviewed against international guidelines, as well as local guidelines/consensus used in Australia, Japan, and China. Suggestions are provided for improving the time to diagnosis in the region, increasing access to guideline-recommended treatments, and providing support to reduce the burden on patients and caregivers. There is an urgent need to improve HAE services and provide access to life-saving treatment in developing countries, and efforts should be made to increase awareness of guideline recommendations in high-income economies that do not currently provide long-term prophylactic treatments. Hereditary angioedema (HAE) due to C1-inhibitor deficiency or dysfunction is a rare genetic disorder that causes recurrent episodes of swelling in various parts of the body. Treatment goals of HAE aim to “normalize” life for all patients; however, lack of diagnostic facilities and limited access to effective treatment options in developing nations cause delays in diagnosis and place a significant burden on patients. In this review, we aim to highlight the burden of disease caused by C1-inhibitor HAE across the Asia-Pacific region, considering its epidemiology, morbidity and mortality, and socioeconomic and psychological impact. We also review the availability of guideline-recommended diagnostic facilities and treatments, and how patients are currently managed. Data were collected from published literature and HAE experts in the region, who provided information regarding diagnosis and management in their countries. Current practice was reviewed against international guidelines, as well as local guidelines/consensus used in Australia, Japan, and China. Suggestions are provided for improving the time to diagnosis in the region, increasing access to guideline-recommended treatments, and providing support to reduce the burden on patients and caregivers. There is an urgent need to improve HAE services and provide access to life-saving treatment in developing countries, and efforts should be made to increase awareness of guideline recommendations in high-income economies that do not currently provide long-term prophylactic treatments. Hereditary angioedema (HAE) due to C1-esterase inhibitor deficiency or dysfunction (C1-INH-HAE) is a rare, potentially life-threatening disease characterized by recurrent acute attacks of skin and/or mucosal edema.1Maurer M. Magerl M. Betschel S. Aberer W. Ansotegui I.J. Aygören-Pürsün E. et al.The international WAO/EAACI guideline for the management of hereditary angioedema—the 2021 revision and update.Allergy. 2022; 77: 1961-1990Crossref PubMed Scopus (120) Google Scholar,2Jindal A.K. Reshef A. Longhurst H. Global Equity in HAE Management WorkgroupMitigating disparity in health-care resources between countries for management of hereditary angioedema.Clin Rev Allergy Immunol. 2021; 61: 84-97Crossref PubMed Scopus (17) Google Scholar The disease is primarily driven by deficiency or dysfunction in C1-INH protein (HAE types 1 and 2, respectively), which leads to dysregulation of the kallikrein-kinin pathway and overproduction of bradykinin, a potent mediator of the inflammatory cascade responsible for vasodilation and vascular permeability.1Maurer M. Magerl M. Betschel S. Aberer W. Ansotegui I.J. Aygören-Pürsün E. et al.The international WAO/EAACI guideline for the management of hereditary angioedema—the 2021 revision and update.Allergy. 2022; 77: 1961-1990Crossref PubMed Scopus (120) Google Scholar,2Jindal A.K. Reshef A. Longhurst H. Global Equity in HAE Management WorkgroupMitigating disparity in health-care resources between countries for management of hereditary angioedema.Clin Rev Allergy Immunol. 2021; 61: 84-97Crossref PubMed Scopus (17) Google Scholar C1-INH-HAE is a chronic disorder with high morbidity and negative impact on patient quality of life (QoL) because of repeated attacks of angioedema, which may be fatal when localized to the larynx.3Banerji A. Davis K.H. Brown et burden of hereditary from a patient in the Allergy Immunol. PubMed Scopus Google Scholar access to effective acute and prophylactic treatments, caused by is A. M. Hereditary and C1-inhibitor and in PubMed Scopus Google Scholar effective in were A. M. Hereditary and C1-inhibitor and in PubMed Scopus Google Scholar which in developing of who from C1-INH-HAE in a was S. The of hereditary angioedema in a PubMed Scopus Google Scholar because access to guideline-recommended in C1-INH-HAE the this is limited to high-income A.K. Reshef A. Longhurst H. Global Equity in HAE Management WorkgroupMitigating disparity in health-care resources between countries for management of hereditary angioedema.Clin Rev Allergy Immunol. 2021; 61: 84-97Crossref PubMed Scopus (17) Google attacks and in hereditary angioedema due to C1-INH Allergy Immunol. PubMed Scopus Google Scholar In developing countries across the Asia-Pacific region, and with guideline-recommended treatment are of A.K. Reshef A. Longhurst H. Global Equity in HAE Management WorkgroupMitigating disparity in health-care resources between countries for management of hereditary angioedema.Clin Rev Allergy Immunol. 2021; 61: 84-97Crossref PubMed Scopus (17) Google Scholar In because of the of treatment as C1-INH or and developing countries to access to guideline-recommended A.K. Reshef A. Longhurst H. 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Aygören-Pürsün E. et al.The international WAO/EAACI guideline for the management of hereditary angioedema—the 2021 revision and update.Allergy. 2022; 77: 1961-1990Crossref PubMed Scopus (120) Google Scholar In to recommendations are also for patients who may be to M. Magerl M. Betschel S. Aberer W. Ansotegui I.J. Aygören-Pürsün E. et al.The international WAO/EAACI guideline for the management of hereditary angioedema—the 2021 revision and update.Allergy. 2022; 77: 1961-1990Crossref PubMed Scopus (120) Google Scholar prophylactic treatment of with HAE is with the to the of angioedema of or with of the M. Magerl M. Betschel S. Aberer W. Ansotegui I.J. Aygören-Pürsün E. et al.The international WAO/EAACI guideline for the management of hereditary angioedema—the 2021 revision and update.Allergy. 2022; 77: 1961-1990Crossref PubMed Scopus (120) Google Scholar The the of as M. Magerl M. Betschel S. Aberer W. Ansotegui I.J.

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