Jae-Woo Jung1,2, Dong In Suh3, Hye Jung Park4, Sujeong Kim5, Hyouk Soo Kwon6, Min Suk Yang7, Chan Sun Park8, Joo-Hee Kim9, Sae-Hoon Kim10, Yong Won Lee11, Gyu Young Hur12, Young-Min Ye13, Yong Eun Kwon14, Hye-Kyung Park15, Cheol Woo Kim16, Young-Il Koh17, Jung Wong Park18, Jong-Myung Lee5, Kyung-Up Min1,19, Paige Wickner20, Hye-Ryun Kang1,19. 1. Institute of Allergy and Clinical Immunology, Seoul National University Medical Research Center, Seoul, Republic of Korea. 2. Department of Internal Medicine, Chung-Ang University College of Medicine, Seoul, Republic of Korea. 3. Department of Pediatrics, Seoul National University College of Medicine, Seoul, Republic of Korea. 4. Department of Internal Medicine, Gangnam Severance Hospital, Yonsei University College of Medicine, Seoul, Republic of Korea. 5. Department of Internal Medicine, School of Medicine, Kyungpook National University, Daegu, Republic of Korea. 6. Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Republic of Korea. 7. Department of Internal Medicine, SMG-SNU Boramae Medical Center, Seoul, Republic of Korea. 8. Department of Internal Medicine, Inje University Haeundae Paik Hospital, Busan, Republic of Korea. 9. Department of Internal Medicine, Hallym University Sacred Heart Hospital, Anyang, Republic of Korea. 10. Department of Internal Medicine, Seoul National University Bundang Hospital, Seongnam, Republic of Korea. 11. Department of Internal Medicine, Catholic Kwandong University College of Medicine, Incheon, Republic of Korea. 12. Department of Internal Medicine, Korea University Guro Hospital, Seoul, Republic of Korea. 13. Department of Internal Medicine, Ajou University School of Medicine, Suwon, Republic of Korea. 14. Department of Internal Medicine, Chosun University Hospital, Gwangju, Republic of Korea. 15. Department of Internal Medicine, Pusan National University School of Medicine, Busan, Republic of Korea. 16. Department of Internal Medicine, Inha University Hospital, Incheon, Republic of Korea. 17. Department of Internal Medicine, Chonnam National University Medical School, Gwangju, Republic of Korea. 18. Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Republic of Korea. 19. Department of Internal Medicine, Seoul National University College of Medicine, Seoul, Republic of Korea. 20. Department of Rheumatology Allergy and Immunology, Brigham and Women's Hospital, Boston, Massachusetts, USA.
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.
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.
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