Official reprint from UpToDate®
www.uptodate.com ©2017 UpToDate, Inc. and/or its affiliates. All Rights Reserved.

Hereditary angioedema: Temporary prophylaxis before procedures or stress to prevent angioedema episodes

Marco Cicardi, MD
Bruce Zuraw, MD
Section Editor
Sarbjit Saini, MD
Deputy Editor
Anna M Feldweg, MD


Hereditary angioedema (HAE) is a rare genetic disorder characterized by recurrent angioedema without wheals [1]. All forms of HAE are characterized by recurrent episodes of angioedema without pruritus or urticaria, which most often affect the skin or mucosal tissues of the upper respiratory and gastrointestinal tracts. Although swelling resolves spontaneously in two to four days in the absence of treatment, angioedema can often be temporarily debilitating, and laryngeal angioedema may cause fatal asphyxiation. There are several different forms of HAE. The recommendations in this topic apply to those due to C1 inhibitor (C1INH) deficiency.

Dental and medical procedures can trigger episodes of angioedema. The various prophylactic therapies that are used prior to dental and medical procedures to prevent attacks will be reviewed here. Long-term prophylaxis to reduce the frequency and severity of angioedema episodes and other issues in the diagnosis and management of HAE are discussed elsewhere:

(See "Hereditary angioedema: General care and long-term prophylaxis".)

(See "Hereditary angioedema: Treatment of acute attacks".)

(See "Hereditary angioedema: Epidemiology, clinical manifestations, exacerbating factors, and prognosis".)

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:

Subscribers log in here

Literature review current through: Sep 2017. | This topic last updated: Sep 29, 2017.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2017 UpToDate, Inc.
  1. Cicardi M, Aberer W, Banerji A, et al. Classification, diagnosis, and approach to treatment for angioedema: consensus report from the Hereditary Angioedema International Working Group. Allergy 2014; 69:602.
  2. Bhardwaj N, Craig TJ. Treatment of hereditary angioedema: a review (CME). Transfusion 2014; 54:2989.
  3. Zuraw BL. Clinical practice. Hereditary angioedema. N Engl J Med 2008; 359:1027.
  4. Magerl M, Frank M, Lumry W, et al. Short-term prophylactic use of C1-inhibitor concentrate in hereditary angioedema: Findings from an international patient registry. Ann Allergy Asthma Immunol 2017; 118:110.
  5. Farkas H, Zotter Z, Csuka D, et al. Short-term prophylaxis in hereditary angioedema due to deficiency of the C1-inhibitor--a long-term survey. Allergy 2012; 67:1586.
  6. Bork K, Hardt J, Staubach-Renz P, Witzke G. Risk of laryngeal edema and facial swellings after tooth extraction in patients with hereditary angioedema with and without prophylaxis with C1 inhibitor concentrate: a retrospective study. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2011; 112:58.
  7. Frank MM. 8. Hereditary angioedema. J Allergy Clin Immunol 2008; 121:S398.
  8. Caballero T, Farkas H, Bouillet L, et al. International consensus and practical guidelines on the gynecologic and obstetric management of female patients with hereditary angioedema caused by C1 inhibitor deficiency. J Allergy Clin Immunol 2012; 129:308.
  9. Bowen T, Cicardi M, Farkas H, et al. 2010 International consensus algorithm for the diagnosis, therapy and management of hereditary angioedema. Allergy Asthma Clin Immunol 2010; 6:24.
  10. Gompels MM, Lock RJ, Abinun M, et al. C1 inhibitor deficiency: consensus document. Clin Exp Immunol 2005; 139:379.
  11. Farkas H, Kohalmi KV, Veszeli N, et al. Successful prophylaxis with recombinant human C1 inhibitor in a patient with hereditary angioedema. Ann Allergy Asthma Immunol 2015; 114:64.
  12. Grant JA, White MV, Li HH, et al. Preprocedural administration of nanofiltered C1 esterase inhibitor to prevent hereditary angioedema attacks. Allergy Asthma Proc 2012; 33:348.
  13. Leimgruber A, Jaques WA, Spaeth PJ. Hereditary angioedema: uncomplicated maxillofacial surgery using short-term C1 inhibitor replacement therapy. Int Arch Allergy Immunol 1993; 101:107.
  14. Langston D, Weiner J, Fary W. C1-esterase inhibitor concentrate prevents upper airways obstruction in hereditary angio-oedema. Med J Aust 1994; 164:383.
  15. Bernstein JA, Ritchie B, Levy RJ, et al. Population pharmacokinetics of plasma-derived C1 esterase inhibitor concentrate used to treat acute hereditary angioedema attacks. Ann Allergy Asthma Immunol 2010; 105:149.
  16. Martinez-Saguer I, Rusicke E, Aygören-Pürsün E, et al. Pharmacokinetic analysis of human plasma-derived pasteurized C1-inhibitor concentrate in adults and children with hereditary angioedema: a prospective study. Transfusion 2010; 50:354.
  17. Cicardi M, Zingale L. How do we treat patients with hereditary angioedema. Transfus Apher Sci 2003; 29:221.
  18. Ott HW, Mattle V, Hadziomerovic D, et al. Treatment of hereditary angioneurotic oedema (HANE) with tibolone. Clin Endocrinol (Oxf) 2007; 66:180.
  19. FOX M, MINOT AS, LIDDLE GW. Oxandrolone: a potent anabolic steroid of novel chemical configuration. J Clin Endocrinol Metab 1962; 22:921.
  20. Church JA. Oxandrolone treatment of childhood hereditary angioedema. Ann Allergy Asthma Immunol 2004; 92:377.
  21. Sheffer AL, Fearon DT, Austen KF. Methyltestosterone therapy in hereditary angioedema. Ann Intern Med 1977; 86:306.
  22. Farkas H, Gyeney L, Gidófalvy E, et al. The efficacy of short-term danazol prophylaxis in hereditary angioedema patients undergoing maxillofacial and dental procedures. J Oral Maxillofac Surg 1999; 57:404.
  23. Hardie J, Ringland T, Yang WH, Wagner V. Potentially fatal hereditary angioedema: a review and case report. J Can Dent Assoc 1990; 56:1096.
  24. Sheffer AL, Fearon DT, Austen KF. Clinical and biochemical effects of stanozolol therapy for hereditary angioedema. J Allergy Clin Immunol 1981; 68:181.
  25. Prematta M, Gibbs JG, Pratt EL, et al. Fresh frozen plasma for the treatment of hereditary angioedema. Ann Allergy Asthma Immunol 2007; 98:383.
  26. Atkinson JC, Frank MM. Oral manifestations and dental management of patients with hereditary angioedema. J Oral Pathol Med 1991; 20:139.
  27. Degroote DF, Smith GL, Huttula GS. Acute airway obstruction following tooth extraction in hereditary angioedema. J Oral Maxillofac Surg 1985; 43:52.
  28. Phillips KM, Glick M, Cohen SG. Hereditary angioedema: report of case. Spec Care Dentist 1989; 9:23.
  29. Cicardi M, Bork K, Caballero T, et al. 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.
  30. Bowen T, Brosz J, Brosz K, et al. Management of hereditary angioedema: 2010 Canadian approach. Allergy Asthma Clin Immunol 2010; 6:20.
  31. Zuraw BL, Bernstein JA, Lang DM, et al. A focused parameter update: hereditary angioedema, acquired C1 inhibitor deficiency, and angiotensin-converting enzyme inhibitor-associated angioedema. J Allergy Clin Immunol 2013; 131:1491.
  32. Zuraw BL, Banerji A, Bernstein JA, et al. US Hereditary Angioedema Association Medical Advisory Board 2013 recommendations for the management of hereditary angioedema due to C1 inhibitor deficiency. J Allergy Clin Immunol Pract 2013; 1:458.
  33. Bork K. An evidence based therapeutic approach to hereditary and acquired angioedema. Curr Opin Allergy Clin Immunol 2014; 14:354.
  34. Betschel S, Badiou J, Binkley K, et al. Canadian hereditary angioedema guideline. Allergy Asthma Clin Immunol 2014; 10:50.