International Archives of Allergy and Immunology

Basic and Clinical Immunology - Original Paper

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

Jung J.-W.a,b · Suh D.I.c · Park H.J.d · Kim S.e · Kwon H. S.f · Yang M. S.g · Park C.S.h · Kim J.-H.i · Kim S.-H.j · Lee Y.W.k · Hur G.Y.l · Ye Y.-M.m · Kwon Y.E.n · Park H.-K.o · Kim C.W.p · Koh Y.-I.q · Park J.W.r · Lee J.-M.e · Min K.-U.a,s · Wickner P.t · Kang H.-R.a,s

Author affiliations

aInstitute of Allergy and Clinical Immunology, Seoul National University Medical Research Center, Seoul, Republic of Korea
bDepartment of Internal Medicine, Chung-Ang University College of Medicine, Seoul, Republic of Korea
cDepartment of Pediatrics, Seoul National University College of Medicine, Seoul, Republic of Korea
dDepartment of Internal Medicine, Gangnam Severance Hospital, Yonsei University College of Medicine, Seoul, Republic of Korea
eDepartment of Internal Medicine, School of Medicine, Kyungpook National University, Daegu, Republic of Korea
fDepartment of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Republic of Korea
gDepartment of Internal Medicine, SMG-SNU Boramae Medical Center, Seoul, Republic of Korea
hDepartment of Internal Medicine, Inje University Haeundae Paik Hospital, Busan, Republic of Korea
iDepartment of Internal Medicine, Hallym University Sacred Heart Hospital, Anyang, Republic of Korea
jDepartment of Internal Medicine, Seoul National University Bundang Hospital, Seongnam, Republic of Korea
kDepartment of Internal Medicine, Catholic Kwandong University College of Medicine, Incheon, Republic of Korea
lDepartment of Internal Medicine, Korea University Guro Hospital, Seoul, Republic of Korea
mDepartment of Internal Medicine, Ajou University School of Medicine, Suwon, Republic of Korea
nDepartment of Internal Medicine, Chosun University Hospital, Gwangju, Republic of Korea
oDepartment of Internal Medicine, Pusan National University School of Medicine, Busan, Republic of Korea
pDepartment of Internal Medicine, Inha University Hospital, Incheon, Republic of Korea
qDepartment of Internal Medicine, Chonnam National University Medical School, Gwangju, Republic of Korea
rDepartment of Internal Medicine, Yonsei University College of Medicine, Seoul, Republic of Korea
sDepartment of Internal Medicine, Seoul National University College of Medicine, Seoul, Republic of Korea
tDepartment of Rheumatology Allergy and Immunology, Brigham and Women’s Hospital, Boston, MA, USA

Keywords: AngioedemaComplement C1s esterase inhibitor proteinsHereditary angioedema types I and IIKoreansMortality

Related Articles for ""
Int Arch Allergy Immunol 2018;176:272–279
https://doi.org/10.1159/000488350

Log in to MyKarger to check if you already have access to this content.




Forgot your password
Sign up to MyKarger

Buy

  • FullText & PDF
  • Unlimited re-access via MyKarger
  • Unrestricted printing, no saving restrictions for personal use
read more
CHF 38.00 *
EUR 35.00 *
USD 39.00 *

Select

KAB

Buy a Karger Article Bundle (KAB) and profit from a discount!


If you would like to redeem your KAB credit, please log in.

Save over 20% compared to the individual article price.

Learn more

Rent/Cloud

  • Rent for 48h to view
  • Buy Cloud Access for unlimited viewing via different devices
  • Synchronizing in the ReadCube Cloud
  • Printing and saving restrictions apply
Rental: USD 8.50
Cloud: USD 20.00

Select

Subscribe

  • Access to all articles of the subscribed year(s) guaranteed for 5 years
  • Unlimited re-access via Subscriber Login or MyKarger
  • Unrestricted printing, no saving restrictions for personal use
read more

Subcription rates

Select
* The final prices may differ from the prices shown due to specifics of VAT rules.

Article / Publication Details

First-Page Preview
Abstract of Basic and Clinical Immunology - Original Paper

Received: October 26, 2017
Accepted: March 13, 2018
Published online: April 25, 2018
Issue release date: July 2018

Number of Print Pages: 8
Number of Figures: 3
Number of Tables: 1

ISSN: 1018-2438 (Print)
eISSN: 1423-0097 (Online)

For additional information: https://www.karger.com/IAA

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.

© 2018 S. Karger AG, Basel



Related Articles:

References

  1. Agostoni A, Aygören-Pürsün E, Binkley KE, Blanch A, Bork K, Bouillet L, Bucher C, Castaldo AJ, Cicardi M, Davis AE, De Carolis C, Drouet C, Duponchel C, Farkas H, Fáy K, Fekete B, Fischer B, Fontana L, Füst G, Giacomelli R, Gröner A, Hack CE, Harmat G, Jakenfelds J, Juers M, Kalmár L, Kaposi PN, Karádi I, Kitzinger A, Kollár T, Kreuz W, Lakatos P, Longhurst HJ, Lopez-Trascasa M, Martinez-Saguer I, Monnier N, Nagy I, Németh E, Nielsen EW, Nuijens JH, O’grady C, Pappalardo E, Penna V, Perricone C, Perricone R, Rauch U, Roche O, Rusicke E, Späth PJ, Szendei G, Takács E, Tordai A, Truedsson L, Varga L, Visy B, Williams K, Zanichelli A, Zingale L: Hereditary and acquired angioedema: problems and progress: Proceedings of the Third C1 Esterase Inhibitor Deficiency Workshop and beyond. J Allergy Clin Immunol 2004; 114:S51–S131.
    External Resources
  2. Roche O, Blanch A, Caballero T, Sastre N, Callejo D, Lopez-Trascasa M: Hereditary angioedema due to C1 inhibitor deficiency: patient registry and approach to the prevalence in Spain. Ann Allergy Asthma Immunol 2005; 94: 498–503.
    External Resources
  3. Bygum A: Hereditary angio-oedema in Denmark: a nationwide survey. Br J Dermatol 2009; 161: 1153–1158.
    External Resources
  4. Longhurst H, Cicardi M: Hereditary angio-oedema. Lancet 2012; 379: 474–481.
    External Resources
  5. Iwamoto K, Mihara S, Ikezawa Z, Hide M: National prevalence survey of hereditary angioedema in Japan. Arerugi 2011; 60: 26–32.
    External Resources
  6. Lei WT, Shyur SD, Huang LH, Kao YH, Lo CY: Type I hereditary angioedema in Taiwan – clinical, biological features and genetic study. Asian Pac J Allergy Immunol 2011; 29: 327–331.
    External Resources
  7. Ren HL, Zhang HY: Clinical features of hereditary angioedema: analysis of 133 cases. Zhonghua Yi Xue Za Zhi 2007; 87: 2772–2776.
    External Resources
  8. Lee JH, Cho JY, Nam DH, Hong CS: A case of hereditary angioedema. Korean J Allergy 1994; 14: 695–701.
  9. Lee YS, Chung JH, Cho KH, Youn JI: A case of hereditary angioedema. Korean J Dermatol 1994; 32: 115–118.
  10. Suh KS, Kang JM, Kim KJ, Kang HJ: Three cases of hereditary angioedema in one family. Korean J Dermatol 1995; 33: 564–569.
  11. Lee JA, Nah BG, Jun H, Seo JC, Kim MK: A case of hereditary angioedema not manifestated classical autosomal dominant trait. Korean J Allergy 1997; 17: 574–579.
  12. Kim SH, Lee BJ, Chang YS, Kim YK, Cho SH, Min KU, Kim YY: A case of hereditary angio-edema associated with idiopathic hypoparathyroidism. Korean J Intern Med 2001; 16: 281–283.
    External Resources
  13. Lee SY, Lee SE, Song WJ, Kang HR, Min KU: A case of cesarean section delivery in a patient with hereditary angioedema. Korean J Asthma Allergy Clin Immunol 2012; 32: 264–267.
  14. Kang HR, Yim EY, Oh SY, Chang YS, Kim YK, Cho SH, Min KU, Kim YY: Normal C1 inhibitor mRNA expression level in type I hereditary angioedema patients: newly found C1 inhibitor gene mutations. Allergy 2006; 61: 260–264.
    External Resources
  15. Shin M, Ahn K: A case of hereditary angioedema in a 7-year-old Korean girl. Allergy Asthma Immunol Res 2013; 5: 59–61.
    External Resources
  16. Lim YC, Kim JK, Shim DB, Lim CH, Jang HJ, Shin HA, Choi EC: Hereditary angioneurotic edema of the larynx. Acta Otolaryngol 2005; 125: 1240–1243.
    External Resources
  17. Chung JY, Kim M: Migraine-like headache in a patient with complement 1 inhibitor-deficient hereditary angioedema. J Korean Med Sci 2012; 27: 104–106.
    External Resources
  18. Sim DW, Park KH, Lee JH, Park JW: A case of type 2 hereditary angioedema with SERPING1 mutation. Allergy Asthma Immunol Res 2017; 9: 96–98.
    External Resources
  19. Lee SY, Kang HR, Jung JW, Jang GC, Lee SY, Ahn YM, Min KU: Clinical experience in managing patients with hereditary angioedema in Korea: questionnaire survey and a literature review. Allergy Asthma Respir Dis 2014; 2: 277–284.
  20. Lunn ML, Santos CB, Craig TJ: Is there a need for clinical guidelines in the United States for the diagnosis of hereditary angioedema and the screening of family members of affected patients? Ann Allergy Asthma Immunol 2010; 104: 211–214.
    External Resources
  21. Frank MM, Gelfand JA, Atkinson JP: Hereditary angioedema: the clinical syndrome and its management. Ann Intern Med 1976; 84: 580–593.
    External Resources
  22. Winnewisser J, Rossi M, Spath P, Burgi H: Type I hereditary angio-oedema. Variability of clinical presentation and course within two large kindreds. J Intern Med 1997; 241: 39–46.
    External Resources
  23. Bork K, Staubach P, Eckardt AJ, Hardt J: Symptoms, course, and complications of abdominal attacks in hereditary angioedema due to C1 inhibitor deficiency. Am J Gastroenterol 2006; 101: 619–627.
    External Resources
  24. Bowen T, Cicardi M, Bork K, Zuraw B, Frank M, Ritchie B, Farkas H, Varga L, Zingale LC, Binkley K, Wagner E, Adomaitis P, Brosz K, Burnham J, Warrington R, Kalicinsky C, Mace S, McCusker C, Schellenberg R, Celeste L, Hebert J, Valentine K, Poon MC, Serushago B, Neurath D, Yang W, Lacuesta G, Issekutz A, Hamed A, Kamra P, Dean J, Kanani A, Stark D, Rivard GE, Leith E, Tsai E, Waserman S, Keith PK, Page D, Marchesin S, Longhurst HJ, Kreuz W, Rusicke E, Martinez- Saguer I, Aygören-Pürsün E, Harmat G, Füst G, Li H, Bouillet L, Caballero T, Moldovan D, Späth PJ, Smith-Foltz S, Nagy I, Nielsen EW, Bucher C, Nordenfelt P, Xiang ZY: Hereditary angiodema: a current state-of-the-art review, VII: Canadian Hungarian 2007 International Consensus Algorithm for the Diagnosis, Therapy, and Management of Hereditary Angioedema. Ann Allergy Asthma Immunol 2008; 100:S30–S40.
  25. Stray-Pedersen A, Abrahamsen TG, Froland SS: Primary immunodeficiency diseases in Norway. J Clin Immunol 2000; 20: 477–485.
    External Resources
  26. Starsia Z, Hegyi E, Kuklinek P, Cap J, Stefanovic J, Lokaj J: Hereditary angioedema in Czechoslovakia. Clinical, immunological, genetic and therapeutic studies of 16 families. Allerg Immunol (Leipz) 1988; 34: 35–42.
    External Resources
  27. Agostoni A, Cicardi M: Hereditary and acquired C1-inhibitor deficiency: biological and clinical characteristics in 235 patients. Medicine 1992; 71: 206–215.
    External Resources
  28. Zilberberg MD, Nathanson BH, Jacobsen T, Tillotson G: Descriptive epidemiology of hereditary angioedema emergency department visits in the United States, 2006–2007. Allergy Asthma Proc 2011; 32: 390–394.
    External Resources
  29. Kodama J, Uchida K, Yoshimura S, Katayama Y, Kushiro H, Yutani C, Funahashi S, Takamiya O, Matsumoto Y, Ando Y, et al: Studies of four Japanese families with hereditary angioneurotic edema: simultaneous activation of plasma protease systems and exogenous triggering stimuli. Blut 1984; 49: 405–418.
    External Resources
  30. Bork K, Meng G, Staubach P, Hardt J: Hereditary angioedema: new findings concerning symptoms, affected organs, and course. Am J Med 2006; 119: 267–274.
    External Resources
  31. Cicardi M, Bergamaschini L, Marasini B, Boccassini G, Tucci A, Agostoni A: Hereditary angioedema: an appraisal of 104 cases. Am J Med Sci 1982; 284: 2–9.
    External Resources
  32. Yamamoto T, Horiuchi T, Miyahara H, Yoshizawa S, Maehara J, Shono E, Taamura K, Machida H, Tsujioka K, Kaneko T, Uemura N, Suzawa K, Inagaki N, Umegaki N, Kasamatsu Y, Hara A, Arinobu Y, Inoue Y, Niiro H, Kashiwagai Y, Harashima S, Tahira T, Tsukamoto H, Akashi K: Hereditary angioedema in Japan: genetic analysis of 13 unrelated cases. Am J Med Sci 2012; 343: 210–214.
    External Resources
  33. Xu YY, Jiang Y, Zhi YX, Yin J, Wang LL, Wen LP, Gu JQ, Guan K, Zhang HY: Clinical features of hereditary angioedema in Chinese patients: new findings and differences from other populations. Eur J Dermatol 2013; 23: 500–504.
    External Resources
  34. Kohler PF, Percy J, Campion WM, Smyth CJ: Hereditary angioedema and “familial” lupus erythematosus in identical twin boys. Am J Med 1974; 56: 406–411.
    External Resources
  35. Banerji A: The burden of illness in patients with hereditary angioedema. Ann Allergy Asthma Immunol 2013; 111: 329–336.
    External Resources
  36. Davis AE 3rd: The pathophysiology of hereditary angioedema. Clin Immunol 2005; 114: 3–9.
    External Resources
  37. Bork K, Hardt J, Witzke G: Fatal laryngeal attacks and mortality in hereditary angioedema due to C1-INH deficiency. J Allergy Clin Immunol 2012; 130: 692–697.
    External Resources
  38. Kim SJ, Brooks JC, Sheikh J, Kaplan MS, Goldberg BJ: Angioedema deaths in the United States. Ann Allergy Asthma Immunol 2014; 113: 630–634.
    External Resources
  39. Ohsawa I, Honda D, Nagamachi S, Hisada A, Shimamoto M, Inoshita H, Mano S, Tomino Y: Clinical manifestations, diagnosis, and treatment of hereditary angioedema: survey data from 94 physicians in Japan. Ann Allergy Asthma Immunol 2015; 114: 492–498.
    External Resources
  40. Cicardi M, Bork K, Caballero T, Craig T, Li HH, Longhurst H, Reshef A, Zuraw B; HAWK (Hereditary Angioedema International Working Group): Evidence-based recommendations for the therapeutic management of angioedema owing to hereditary C1 inhibitor deficiency: consensus report of an International Working Group. Allergy 2012; 67: 147–157.
    External Resources
  41. Nzeako UC, Frigas E, Tremaine WJ: Hereditary angioedema: a broad review for clinicians. Arch Intern Med 2001; 161: 2417–2429.
    External Resources

Article / Publication Details

First-Page Preview
Abstract of Basic and Clinical Immunology - Original Paper

Received: October 26, 2017
Accepted: March 13, 2018
Published online: April 25, 2018
Issue release date: July 2018

Number of Print Pages: 8
Number of Figures: 3
Number of Tables: 1

ISSN: 1018-2438 (Print)
eISSN: 1423-0097 (Online)

For additional information: https://www.karger.com/IAA

Copyright / Drug Dosage / Disclaimer

Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.

Article Tools
Article Details

2018, Vol.176, No. 3-4

July 2018

Article

Recommend This
Additional Information

Edited by: W.F. Pickl, Vienna.

TOP