2010 International consensus algorithm for the diagnosis, therapy and management of hereditary angioedema

Tom Bowen, Marco Cicardi, Henriette Farkas, Konrad Bork, Hilary J Longhurst, Bruce Zuraw, Emel Aygoeren-Pürsün, Timothy Craig, Karen Binkley, Jacques Hebert, Bruce Ritchie, Laurence Bouillet, Stephen Betschel, Della Cogar, John Dean, Ramachand Devaraj, Azza Hamed, Palinder Kamra, Paul K Keith, Gina Lacuesta, Eric Leith, Harriet Lyons, Sean Mace, Barbara Mako, Doris Neurath, Man-Chiu Poon, Georges-Etienne Rivard, Robert Schellenberg, Dereth Rowan, Anne Rowe, Donald Stark, Smeeksha Sur, Ellie Tsai, Richard Warrington, Susan Waserman, Rohan Ameratunga, Jonathan Bernstein, Janne Björkander, Kristylea Brosz, John Brosz, Anette Bygum, Teresa Caballero, Mike Frank, George Fust, George Harmat, Amin Kanani, Wolfhart Kreuz, Marcel Levi, Henry Li, Inmaculada Martinez-Saguer, Dumitru Moldovan, Istvan Nagy, Erik W Nielsen, Patrik Nordenfelt, Avner Reshef, Eva Rusicke, Sarah Smith-Foltz, Peter Späth, Lilian Varga, Zhi Yu Xiang, Tom Bowen, Marco Cicardi, Henriette Farkas, Konrad Bork, Hilary J Longhurst, Bruce Zuraw, Emel Aygoeren-Pürsün, Timothy Craig, Karen Binkley, Jacques Hebert, Bruce Ritchie, Laurence Bouillet, Stephen Betschel, Della Cogar, John Dean, Ramachand Devaraj, Azza Hamed, Palinder Kamra, Paul K Keith, Gina Lacuesta, Eric Leith, Harriet Lyons, Sean Mace, Barbara Mako, Doris Neurath, Man-Chiu Poon, Georges-Etienne Rivard, Robert Schellenberg, Dereth Rowan, Anne Rowe, Donald Stark, Smeeksha Sur, Ellie Tsai, Richard Warrington, Susan Waserman, Rohan Ameratunga, Jonathan Bernstein, Janne Björkander, Kristylea Brosz, John Brosz, Anette Bygum, Teresa Caballero, Mike Frank, George Fust, George Harmat, Amin Kanani, Wolfhart Kreuz, Marcel Levi, Henry Li, Inmaculada Martinez-Saguer, Dumitru Moldovan, Istvan Nagy, Erik W Nielsen, Patrik Nordenfelt, Avner Reshef, Eva Rusicke, Sarah Smith-Foltz, Peter Späth, Lilian Varga, Zhi Yu Xiang

Abstract

Background: We published the Canadian 2003 International Consensus Algorithm for the Diagnosis, Therapy, and Management of Hereditary Angioedema (HAE; C1 inhibitor [C1-INH] deficiency) and updated this as Hereditary angioedema: a current state-of-the-art review: Canadian Hungarian 2007 International Consensus Algorithm for the Diagnosis, Therapy, and Management of Hereditary Angioedema.

Objective: To update the International Consensus Algorithm for the Diagnosis, Therapy and Management of Hereditary Angioedema (circa 2010).

Methods: The Canadian Hereditary Angioedema Network (CHAEN)/Réseau Canadien d'angioédème héréditaire (RCAH) http://www.haecanada.com and cosponsors University of Calgary and the Canadian Society of Allergy and Clinical Immunology (with an unrestricted educational grant from CSL Behring) held our third Conference May 15th to 16th, 2010 in Toronto Canada to update our consensus approach. The Consensus document was reviewed at the meeting and then circulated for review.

Results: This manuscript is the 2010 International Consensus Algorithm for the Diagnosis, Therapy and Management of Hereditary Angioedema that resulted from that conference.

Conclusions: Consensus approach is only an interim guide to a complex disorder such as HAE and should be replaced as soon as possible with large phase III and IV clinical trials, meta analyses, and using data base registry validation of approaches including quality of life and cost benefit analyses, followed by large head-to-head clinical trials and then evidence-based guidelines and standards for HAE disease management.

Figures

Figure 1
Figure 1
Hereditary Angioedema - HAE - Diagnostic Algorithm.
Figure 2
Figure 2
Hereditary Angioedema - HAE - Prophylaxis Algorithm.
Figure 3
Figure 3
Treatment of Acute Hereditary Angioedema - HAE - Attacks.

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