Abstract
Background: Hereditary angioedema (HAE) is a rare and life-threatening genetic disease. This disease can lead to recurrent attacks of severe swelling occurring in areas such as the face, gastrointestinal tract, and larynx. These attacks can result in hospitalization or death. Thus, effective medications are necessary for treating the acute attacks. However, Thailand did not specify medication for treatment and prophylaxis. Therefore, effective strategies for universal health coverage for the management of HAE patients remain a challenge in Thailand. This review aims to determine the extent of research in the published literature that examine benefit package and strategies for universal health coverage for the management of HAE patients in Thailand. Method: On 5th January 2022, Embase, Scopus, OpenGrey, PubMed, Science direct, Clinicaltrials.gov, and Cochrane were searched for literature published from inception. Studies evaluating the efficacy and safety of medication for treating acute attacks in HAE patients were included. Additionally, this study performed a survey of data from the drug company, experts in the field, and stakeholders. Our survey asked about the situation, cost, and health service systems for screening, diagnosis, treatment, and referring HAE patients in Thailand. Result: Of 4,834 articles identified, 28 studies were included. Twenty-four studies are original articles, one study was a systematic review and network meta-analysis, and three studies were cost-effectiveness analyses. From current trials, first-line therapy such as Plasma-derived C1 inhibitor, Recombinant C1 inhibitor Conestat alfa, Icatibant, and Ecallantide showed benefits over placebo.However, the study related to the full economic evaluation of first-line therapy in Thailand has not been provided. Conclusion: HAE may affect the budget of Thailand if patients do not receive appropriate treatment. Thus, screening, diagnosis, and effective medication for treating acute attacks are essential measures to decrease the overall HAE-related healthcare cost. However, full economic evaluation studies of first-line therapy in Thailand are needed.