Allergy Asthma Respir Dis.
2022 Apr;10(2):80-88. 10.4168/aard.2022.10.2.80.
Diagnosis and treatment of hereditary angioedema: An expert opinion
- Jung JW1
- Park SY1
- Yoon SY2
- Kim GW3
- Sohn KH4
- Kang SY5
- Park HJ6
- Kang MK7
- Kim JH8
- Park KH9,10
- Suh DI11,12
- Lee DH13
- Kim SH14
- Kwon HS15
- Kang HR
16,17
- Affiliations
-
- 1Department of Internal Medicine, Chung-Ang University College of Medicine, Seoul, Korea
- 2Division of Pulmonology and Allergy, Department of Internal Medicine, Chungnam National University Sejong Hospital, Sejong, Korea
- 3Department of Internal Medicine, St. Carollo General Hospital, Suncheon, Korea
- 4Division of Pulmonology and Allergy, Department of Internal Medicine, KyungHee University Medical Center, Seoul, Korea
- 5Division of Pulmonology and Allergy, Department of Internal Medicine, Gachon University Gil Medical Center, Incheon, Korea
- 6Department of Internal Medicine, Gangnam Severance Hospital, Yonsei University College of Medicine, Seoul, Korea
- 7Departmemt of Internal Medicine, Chungbuk National University Hospital, Chungbuk National College of Medicine, Cheongju, Korea
- 8Division of Pulmonary, Allergy, and Critical Care Medicine, Hallym University Sacred Heart Hospital, Hallym University College of Medicine, Anyang, Korea
- 9Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea
- 10Institute for Allergy, Yonsei University College of Medicine, Seoul, Korea
- 11Department of Pediatrics, Seoul National University Hospital, Seoul, Korea
- 12Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea
- 13Department of Dermatology, Seoul National University College of Medicine, Seoul, Korea
- 14Department of Internal Medicine, Seoul National University Bundang Hospital, Seongnam, Korea
- 15Department of Allergy and Clinical Immunology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
- 16Institute of Allergy and Clinical Immunology, Seoul National University Medical Research Center, Seoul, Korea
- 17Department of Internal Medicine, Seoul National University College of Medicine, Seoul, Korea
- KMID: 2529489
- DOI: http://doi.org/10.4168/aard.2022.10.2.80
Abstract
- Hereditary angioedema (HAE) is a rare disease, but it severely interrupts daily life activities and can sometimes be life-threatening. Therefore, early diagnosis and prompt treatment of HAE attacks are critical. Physicians should be aware of how to diagnose and manage HAE to prepare not to miss a diagnosis when treating HAE patients. Physicians must also carry out tests to confirm the diagnosis of HAEs caused by C1 inhibitor deficiency (type 1) or C1 inhibitor dysfunction (type 2) in patients with recurrent angioedema. In addition, recent studies revealed another type of HAE which is not related to C1 inhibitor (normal C1 inhibitor HAE). Once HAE is confirmed, patients and their caregivers should be given with short-term and long-term treatment plans to relieve or prevent HAE attacks. HAE requires life-long measures, including psychological support for patients and self-management education.
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