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

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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/Société d'Angioédème Héréditaire 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.

Section snippets

Consensus algorithm

  • 1.

    Clinical characteristics2

    • a.

      Recurrent angioedema (swelling) without urticaria (without hiving) that is usually nonpruritic (without itch, although sometimes there is a nonpruritic serpentine erythematous rash).22

    • b.

      Swelling can affect the extremities, face, trunk, gastrointestinal tract, genitourinary regions, or upper airways (any area of the body can be involved; Fig 1).

    • c.

      Abdominal symptoms might mimic infantile colic, acute appendicitis, or acute abdomen, and symptoms might include nausea, vomiting,

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  • Kreuz W, Martinez-Saguer I, Aygören-Pürsün E, Rusicke E, Müller W, Klingebiel Th. Clinical management of hereditary...
  • Cited by (0)

    M. Cicardi has consultant arrangements with Dyax, Pharming, Jerini, and Aventis. C. Bucher is a consultant for ZLB Behring (formerly Aventis Behring). W. Kreuz has received grants–research support from Aventis Behring for research in the field of coagulation disorders and immune deficiencies. B. Zuraw has consultant arrangements with Dyax, Lev Pharmaceuticals, Aventis Behring, and Pharming. All other authors—no potential conflicts of interest disclosed.

    On behalf of PREHAEAT, European Concerted Action “Novel methods for predicting, preventing, and treating attacks in patients with hereditary angioedema.” Participants: Marco Cicardi, MD, Milan, Italy; Konrad Bork, MD, Mainz, Germany; Laurence Bouillet, MD, Grenoble, France; Anette Bygum, MD, Odense, Denmark; Henriette Farkas, MD, PhD, Budapest, Hungary; Erik Hack, MD, Amsterdam, The Netherlands; Margarita López-Trascasa, PhD, Madrid, Spain; Erik Waage Nielsen, MD, Bodö, Norway; Christoph Bucher, MD, Zürich, Switzerland; Lennart Truedsson, PhD, Lund, Sweden; and Hilary Longhurst, MD, London, United Kingdom.

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