Canadian 2003 International Consensus Algorithm for the Diagnosis, Therapy, and Management of Hereditary Angioedema

Tom Bowen, Marco Cicardi, Henriette Farkas, Konrad Bork, Wolfhart Kreuz, Lorenza Zingale, Lilian Varga, Inmaculada Martinez-Saguer, Emel Aygören-Pürsün, Karen Binkley, Bruce Zuraw, Alvin Davis, Jacques Hebert, Bruce Ritchie, Jeanne Burnham, Anthony Castaldo, Alejandra Menendez, Istvan Nagy, George Harmat, Christoph BucherGina Lacuesta, Andrew Issekutz, Richard Warrington, William Yang, John Dean, Amin Kanani, Donald Stark, Christine McCusker, Eric Wagner, Georges Etienne Rivard, Eric Leith, Ellie Tsai, Michael MacSween, John Lyanga, Bazir Serushago, Art Leznoff, Susan Waserman, Jean De Serres

Research output: Contribution to journalArticle

181 Citations (Scopus)


C1 inhibitor deficiency (hereditary angioedema [HAE]) is a rare disorder for which there is a lack of consensus concerning diagnosis, therapy, and management, particularly in Canada. European initiatives have driven the approach to managing HAE with 3 C1-INH Deficiency Workshops held every 2 years in Hungary starting in 1999, with the third Workshop having recently been held in May 2003. The European Contact Board has established a European HAE Registry that will hopefully advance our knowledge of this disorder. The Canadian Hereditary Angioedema Society/Socit d'Angiodème Hrditaire du Canada organized a Canadian International Consensus Conference held in Toronto, Ontario, Canada, on October 24 to 26, 2003, to foster consensus between major European and North American HAE treatment centers. Papers were presented by investigators from Europe and North America, and this consensus algorithm approach was discussed. There is a paucity of double-blind placebo-controlled trials in the treatment of HAE, making levels of evidence to support the algorithm less than optimal. Enclosed is the consensus algorithm approach recommended for the diagnosis, therapy, and management of HAE and agreed to by the authors of this article. This document is only a consensus algorithm approach and requires validation. As such, participants agreed to make this a living 2003 algorithm (ie, a work in progress) and agreed to review its content at future international HAE meetings. The consensus, however, has strength in that it was arrived at by the meeting of patient-care providers along with patient group representatives and individual patients reviewing information available to date and reaching agreement on how to approach the diagnosis, therapy, and management of HAE circa 2003. Hopefully evidence to support approaches to the management of HAE will approach the level of meta-analysis of randomized controlled trials in the near future.

Original languageEnglish
Pages (from-to)629-637
Number of pages9
JournalJournal of Allergy and Clinical Immunology
Issue number3
Publication statusPublished - Sep 1 2004


  • C1 inhibitor
  • C1 inhibitor deficiency
  • Hereditary angioedema
  • consensus

ASJC Scopus subject areas

  • Immunology and Allergy
  • Immunology

Fingerprint Dive into the research topics of 'Canadian 2003 International Consensus Algorithm for the Diagnosis, Therapy, and Management of Hereditary Angioedema'. Together they form a unique fingerprint.

  • Cite this

    Bowen, T., Cicardi, M., Farkas, H., Bork, K., Kreuz, W., Zingale, L., Varga, L., Martinez-Saguer, I., Aygören-Pürsün, E., Binkley, K., Zuraw, B., Davis, A., Hebert, J., Ritchie, B., Burnham, J., Castaldo, A., Menendez, A., Nagy, I., Harmat, G., ... De Serres, J. (2004). Canadian 2003 International Consensus Algorithm for the Diagnosis, Therapy, and Management of Hereditary Angioedema. Journal of Allergy and Clinical Immunology, 114(3), 629-637.