Abstract
Hereditary angioedema (HAE) is a rare bradykinin-mediated disease that is characterized by recurrent attacks of subcutaneous or submucosal edema, which can be life threatening. HAE affects all ethnic groups equally and does not differentiate between age, sex, or race. However, the availability of therapies varies amongst countries resulting in a lack of uniformity of care. Not only is there a disparity of medication availability, but since HAE is a rare disease, it is frequently overlooked and the diagnosis is missed. Even with diagnosis, treatment and management is often less than optimal. For these reasons, it is essential to have practice parameters and guidelines. In this chapter, we focus on recent guidelines. These guidelines deal with recognition, diagnosis, medical care, patient management, and assessment, all which are essential to provide optimal care to people with a rare and orphan disease. The intent of the guidelines, and thus this chapter, is to reduce morbidity and mortality, and restore a normal quality of life for the patient with HAE. We will review the guidelines from various regions of the world as well as international group recommendations. In addition, specific patient populations such as the pregnant, elderly, and juvenile require modified treatment regimens, and for this reason, we have included these data as well. The intent of this chapter is to aid the practitioner in holistic care of the patient with HAE in order to ultimately provide the best standard of care possible.
Similar content being viewed by others
References
Gompels MM, Lock RJ, Morgan JE et al (2002) A multicentre evaluation of the diagnostic efficiency of serological investigations for C1 inhibitor deficiency. J Clin Pathol 55:145–147
Bowen T, Hebert J, Ritchie B et al (2003) Management of hereditary angioedema: a Canadian approach. Transfus Apher Sci Off J World Apher Assoc Off J Eur Soc Haemapheresis 29:205–214. doi:10.1016/j.transci.2003.08.009
Agostoni A, Aygören-Pürsün E, Binkley KE et al (2004) Hereditary and acquired angioedema: problems and progress: proceedings of the third C1 esterase inhibitor deficiency workshop and beyond. J Allergy Clin Immunol 114:S51–131. doi:10.1016/j.jaci.2004.06.047
Bowen T, Cicardi M, Farkas H et al (2004) Canadian 2003 International Consensus Algorithm for the Diagnosis, Therapy, and Management of Hereditary Angioedema. J Allergy Clin Immunol 114:629–637. doi:10.1016/j.jaci.2004.06.043
Gompels MM, Lock RJ, Abinun M et al (2005) C1 inhibitor deficiency: consensus document. Clin Exp Immunol 139:379–394. doi:10.1111/j.1365-2249.2005.02726.x
Bowen T, Cicardi M, Bork K et al (2008) 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 Off Publ Am Coll Allergy Asthma Immunol 100:S30–40
Reshef A, Kivity S, Toubi E (2009) Clinical guidelines for the diagnosis and management of hereditary angioedema. Harefuah update. Allergy & Immunology 12–20 [Heb].
Bowen T, Cicardi M, Farkas H et al (2010) 2010 International consensus algorithm for the diagnosis, therapy and management of hereditary angioedema. Allergy Asthma Clin Immunol Off J Can Soc Allergy Clin Immunol 6:24. doi:10.1186/1710-1492-6-24
Longhurst HJ, Farkas H, Craig T et al (2010) HAE international home therapy consensus document. Allergy Asthma Clin Immunol Off J Can Soc Allergy Clin Immunol 6:22. doi:10.1186/1710-1492-6-22
Caballero T, Baeza ML, Cabañas R et al (2011) Consensus statement on the diagnosis, management, and treatment of angioedema mediated by bradykinin. Part II. Treatment, follow-up, and special situations. J Investig Allergol Clin Immunol 21:422–441, quiz 442–443
Caballero T, Baeza ML, Cabañas R et al (2011) Consensus statement on the diagnosis, management, and treatment of angioedema mediated by bradykinin. Part I. Classification, epidemiology, pathophysiology, genetics, clinical symptoms, and diagnosis. J Investig Allergol Clin Immunol 21:333–347, quiz follow 347
Bork K, Maurer M, Bas M, et al. (2011) German Guidelines for hereditary angioedema due to C1-INH deficiency. Available from: www.angioedema.de/englisch/infos.htm. Accessed 25 Dec 2015
Cicardi M, Bork K, Caballero T et al (2012) Evidence-based recommendations for the therapeutic management of angioedema owing to hereditary C1 inhibitor deficiency: consensus report of an International Working Group. Allergy 67:147–157. doi:10.1111/j.1398-9995.2011.02751.x
Craig T, Aygören-Pürsün E, Bork K et al (2012) WAO guideline for the management of hereditary angioedema. World Allergy Organ J 5:182–199. doi:10.1097/WOX.0b013e318279affa
Caballero T, Farkas H, Bouillet L et al (2012) 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 129:308–320. doi:10.1016/j.jaci.2011.11.025
Wahn V, Aberer W, Eberl W et al (2012) Hereditary angioedema (HAE) in children and adolescents-a consensus on therapeutic strategies. Eur J Pediatr 171:1339–1348. doi:10.1007/s00431-012-1726-4
Zuraw BL, Bork K, Binkley KE et al (2012) Hereditary angioedema with normal C1 inhibitor function: consensus of an international expert panel. Allergy Asthma Proc Off J Reg State Allergy Soc 33(Suppl 1):S145–156. doi:10.2500/aap.2012.33.3627
Zuraw BL, Banerji A, Bernstein JA et al (2013) 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 1:458–467. doi:10.1016/j.jaip.2013.07.002
Jaiganesh T, Wiese M, Hollingsworth J et al (2013) Acute angioedema: recognition and management in the emergency department. Eur J Emerg Med Off J Eur Soc Emerg Med 20:10–17. doi:10.1097/MEJ.0b013e328356f76e
Cicardi M, Aberer W, Banerji A et al (2014) Classification, diagnosis, and approach to treatment for angioedema: consensus report from the Hereditary Angioedema International Working Group. Allergy 69:602–616. doi:10.1111/all.12380
Betschel S, Badiou J, Binkley K et al (2014) Canadian hereditary angioedema guideline. Allergy Asthma Clin Immunol Off J Can Soc Allergy Clin Immunol 10:50. doi:10.1186/1710-1492-10-50
Moellman JJ, Bernstein JA, Lindsell C et al (2014) A consensus parameter for the evaluation and management of angioedema in the emergency department. Acad Emerg Med Off J Soc Acad Emerg Med 21:469–484. doi:10.1111/acem.12341
Cicardi M, Bellis P, Bertazzoni G et al (2014) Guidance for diagnosis and treatment of acute angioedema in the emergency department: consensus statement by a panel of Italian experts. Intern Emerg Med 9:85–92. doi:10.1007/s11739-013-0993-z
Longhurst HJ, Tarzi MD, Ashworth F et al (2015) C1 inhibitor deficiency: 2014 United Kingdom consensus document. Clin Exp Immunol 180:475–483. doi:10.1111/cei.12584
Giavina-Bianchi P, França AT, Grumach AS et al (2011) Brazilian guidelines for the diagnosis and treatment of hereditary angioedema. Clin São Paulo Braz 66:1627–1636
Horiuchi T, Ohi H, Ohsawa I et al (2012) Guideline for hereditary angioedema (HAE) 2010 by the Japanese Association for Complement Research—secondary publication. Allergol Int Off J Jpn Soc Allergol 61:559–562. doi:10.2332/allergolint.12-RAI-0471
Prematta MJ, Prematta T, Craig TJ (2008) Treatment of hereditary angioedema with plasma-derived C1 inhibitor. Ther Clin Risk Manag 4:975–982
Kuhlen JL, Banerji A (2015) Hereditary angioedema: special consideration in children, women of childbearing age, and the elderly. Allergy Asthma Proc Off J Reg State Allergy Soc 36:425–432. doi:10.2500/aap.2015.36.3911
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Jose, J., Zacharias, J. & Craig, T. Review of Select Practice Parameters, Evidence-Based Treatment Algorithms, and International Guidelines for Hereditary Angioedema. Clinic Rev Allerg Immunol 51, 193–206 (2016). https://doi.org/10.1007/s12016-016-8546-7
Published:
Issue Date:
DOI: https://doi.org/10.1007/s12016-016-8546-7