Clinical experience in managing patients with hereditary angioedema in Korea: questionnaire survey and a literature review

Suh-Young Lee; Hye-Ryun Kang; Jae Woo Jung; Gwang Cheon Jang; Soo Young Lee; Youngmin Ahn; Kyung-Up Min

doi:10.4168/aard.2014.2.4.277

Journal List > Allergy Asthma Respir Dis > v.2(4) > 1059040

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This article has been corrected. See "ERRATUM: Correction of explanation for footnote in Table 3. Clinical experience in managing patients with hereditary angioedema in Korea: questionnaire survey and a literature review" in Volume 4 on page 230.

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Lee, Kang, Jung, Jang, Lee, Ahn, Min, and Korean Academy of Asthma, Allergy and Clinical Immunology Anaphylaxis Work Group: Clinical experience in managing patients with hereditary angioedema in Korea: questionnaire survey and a literature review

Original Article

Allergy, Asthma & Respiratory Disease 2014; 2(4): 277-284.

Published online: 30 September 2014

DOI: https://doi.org/10.4168/aard.2014.2.4.277

Clinical experience in managing patients with hereditary angioedema in Korea: questionnaire survey and a literature review

Suh-Young Lee¹, Hye-Ryun Kang¹, Jae Woo Jung², Gwang Cheon Jang³, Soo Young Lee⁴, Youngmin Ahn⁵, Kyung-Up Min¹; Korean Academy of Asthma, Allergy and Clinical Immunology Anaphylaxis Work Group^*

¹Department of Internal Medicine, Seoul National University College of Medicine, Seoul, Korea.

²Department of Internal Medicine, Chung-Ang University College of Medicine, Seoul, Korea.

³Department of Pediatrics, National Health Insurance Corporation Ilsan Hospital, Goyang, Korea.

⁴Department of Pediatrics and Adolescent Medicine, Ajou University School of Medicine, Suwon, Korea.

⁵Department of Pediatrics, Eulji University School of Medicine, Daejeon, Korea.

Correspondence to: Hye-Ryun Kang. Department of Internal Medicine, Seoul National University College of Medicine, 103 Daehak-ro, Jongno-gu, Seoul 110-799, Korea. Tel: +82-2-2072-0820, Fax: +82-2-764-2199, helenmed@snu.ac.kr

Co-correspondence to: Kyung-Up Min. Department of Internal Medicine, Seoul National University College of Medicine, 103 Daehak-ro, Jongno-gu, Seoul 110-799, Korea. Tel: +82-2-2072-3286, Fax: +82-2-762-9662, drmin@snu.ac.kr

*The affiliation of members of the Korean Academy of Asthma, Allergy and Clinical Immunology Anaphylaxis Work Group are listed in ACKNOWLEDGMENTS section.

Received 21 January 2014 Revised 18 March 2014 Accepted 22 April 2014

(open-access):

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/).

Abstract

Purpose

Hereditary angioedema is a familial disease which is caused by a genetic deficiency or functional defect of the C1 inhibitor, and it features episodic swelling that can affect any part of the body. A great number of patients are estimated not to have an accurate diagnosis after the onset of symptoms, and close attention is required because sudden hereditary angioedema attacks can result in even death.

Methods

We sent an e-mail questionnaire to 975 members of the Korean Academy of Asthma, Allergy and Clinical Immunology. A total of 82 members replied. The questionnaire, including 15 questions about the diagnosis and management of hereditary angioedema, was developed by the anaphylaxis/urticaria, angioedema workgroup of the Korean Academy of Asthma, Allergy and Clinical Immunology.

Results

Forty-two percent of the respondents had experience with treatment of a suspected case of hereditary angioedema, and 15.9% made a confirmed diagnosis of hereditary angioedema. When the respondents suspected of cases, 91.4% of them performed tests for C3 and C4 concentrations and C1 inhibitor level. For maintenance treatment, most of the respondents used androgen, and only 22% found that C1 inhibitor concentrates can be prescribed through the Korea Orphan Drug Center in Korea.

Conclusion

Allergy physicians in Korea substantially recognized the correct diagnosis and treatment of hereditary angioedema. However, there was a lack of awareness for the latest treatments, such as C1 inhibitor concentrates. Education of doctors and the public is needed.

Keywords: Hereditary angioedema, Questionnaires, Korea, disease management

Figures and Tables

Fig. 1

Age distribution of respondents.

Fig. 2

Diagnostic methods for cases suspected as a hereditary angioedema (multiple responses were allowed). C1 INH, C1 inhibitor.

Fig. 3

Drugs ever used for the maintenance treatment of hereditary angioedema by respondents (multiple responses were allowed). C1 INH, C1 inhibitor; FFP, fresh frozen plasma; EACA, ε-aminocaproic acid.

Fig. 4

Drugs that respondents answered as agents for the acute attack (dark gray) and the maintenance or prophylactic treatment (light gray) (multiple responses were allowed). C1 INH, C1 inhibitor; FFP, fresh frozen plasma; EACA, ε-aminocaproic acid.

Table 1

Comparison of HAE and AAE

HAE, hereditary angioedema; AAE, acquired angioedema; C1 INH, C1 inhibitor.

Table 2

Summary of hereditary angioedema cases reported in Korean

C1 INH, C1 inhibitor; NA, not assessed.

^*Clinical data of individual cases are not included in the reference 21.

Table 3

New treatment options for hereditary angioedema

INH, inhibitor; IV, intravenous; SC, subcutaneous.

^*Only Cinryze is approved for maintenance treatment by Food and Drug Administration. ^†Kallikrein inhibitor. ^‡Bradykinin B2 receptor antagonist.

Table 4

Treatment options for hereditary angioedema available in Korea

KRW, Korean Won (the currency of South Korea); FFP, fresh frozen plasma; INH, inhibitor; IV, intravenous; SC: subcutaneous.

^*Can be varied according to a value of currency. Adapted from http://www.drug info.co.kr.

ACKNOWLEDGMENTS

Hye-Ryun Kang¹, Hye One Kim², Hae-Sim Park³, Meeyong Shin⁴, Young-Min Ye⁵, Soo-Young Lee⁶, Yoon Soo Lee⁷, Gwang Cheon Jang⁸, Jae Woo Jung⁹, Hyun-Jung Jin¹⁰, Sun Hee Choi¹¹, Youngmin Ahn¹²

¹Division of Allergy and Clinical Immunology, Department of Internal Medicine, Seoul National University College of Medicine, Seou; ²Department of Dermatology, Hallym University College of Medicine, Chuncheon; ³Department of Allergy and Clinical Immunology, Ajou University School of Medicine, Suwon; ⁴Department of Pediatrics, Soonchunhyang University College of Medicine, Cheonan; ⁵Department of Allergy and Clinical Immunology, Ajou University School of Medicine, Suwon; ⁶Department of Pediatrics and Adolescent Medicine, Ajou University School of Medicine, Suwon; ⁷Department of Allergy and Clinical Immunology, Sam Medical Center, Anyang; ⁸Department of Pediatirics, National Health Insurance Corporation Ilsan Hospital, Goyang; ⁹Department of Pulmonary, Chung-Ang University College of Medicine, Seoul; ¹⁰Department of Internal Medicine, Yeungnam University College of Medicine, Daegu; ¹¹Department of Pediatrics, Kyung Hee University School of Medicine, Seoul; ¹²Department of Pediatrics, Eulji University School of Medicine, Daejeon, Korea

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