Clinical Features of Hereditary Angioedema in Korean Patients: A Nationwide Multicenter Study

Jae Woo Jung, Dong In Suh, Hye Jung Park, Sujeong Kim, Hyouk Soo Kwon, Min Suk Yang, Chan Sun Park, Joo Hee Kim, Sae Hoon Kim, Yong Won Lee, Gyu Young Hur, Young Min Ye, Yong Eun Kwon, Hye Kyung Park, Cheol Woo Kim, Young Il Koh, Jung Wong Park, Jong Myung Lee, Kyung Up Min, Paige WicknerHye Ryun Kang

    Research output: Contribution to journalArticlepeer-review

    11 Citations (Scopus)

    Abstract

    Background: Hereditary angioedema (HAE) is a genetically heterogeneous autosomal dominant disorder characterized by recurrent episodes of nonpruritic, nonpitting edema increasing after puberty. It can be fatal due to laryngeal or gastrointestinal (GI) involvement with varied and changing frequency of mortality according to studies published from the Western countries. Epidemiological and clinical data of HAE in Asian countries are sparse. We sought to examine the clinical characteristics of HAE patients in Korea. Methods: Patients diagnosed with HAE at 15 tertiary hospitals across the country until 2016 were retrospectively reviewed. Results: A total of 65 patients diagnosed with HAE by 2016 were identified. The prevalence of HAE was estimated at 1.3/1,000,000 in Korea. Of the 65 patients, 21 (32.3%) were males. A total of 90.8% patients had type I HAE, while the remaining 9.2% patients had type II HAE. The first symptom developed after 20 years in 73.8% of patients, with a mean age 28.4 ± 14.1 years. The age at diagnosis was 36.5 ± 15.8 years, with a mean time delay of 7.8 ± 10.5 years. While the face (82.3%) and extremities (upper 71.0%, lower 62.9%) were the most frequently involved, the GI tract was affected in 40.5% of Korean HAE patients. Prophylaxis was maintained in 62.5% of patients. There was no reported case of death from HAE so far. Conclusions: The clinical manifestation and severity of HAE may vary according to ethnicity. HAE is more infrequent and GI involvement is less likely in Korea compared with Western countries.

    Original languageEnglish
    Pages (from-to)272-279
    Number of pages8
    JournalInternational Archives of Allergy and Immunology
    Volume176
    Issue number3-4
    DOIs
    Publication statusPublished - 2018 Jul 1

    Keywords

    • Angioedema
    • Complement C1s esterase inhibitor proteins
    • Hereditary angioedema types I and II
    • Koreans Mortality

    ASJC Scopus subject areas

    • Immunology and Allergy
    • Immunology

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