Canadian 2003 International Consensus Algorithm For the Diagnosis, Therapy, and Management of Hereditary Angioedema.
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.
Bowen
Tom
T
Department of Medicine and Paeditrics, University of Calgary, Calgary, Alberta T2N 2T8, Canada. tbowen@pol.net
Cicardi
Marco
M
Farkas
Henriette
H
Bork
Konrad
K
Kreuz
Wolfhart
W
Zingale
Lorenza
L
Varga
Lilian
L
Martinez-Saguer
Inmaculada
I
Aygören-Pürsün
Emel
E
Binkley
Karen
K
Zuraw
Bruce
B
Davis
Alvin
A
Hebert
Jacques
J
Ritchie
Bruce
B
Burnham
Jeanne
J
Castaldo
Anthony
A
Menendez
Alejandra
A
Nagy
Istvan
I
Harmat
George
G
Bucher
Christoph
C
Lacuesta
Gina
G
Issekutz
Andrew
A
Warrington
Richard
R
Yang
William
W
Dean
John
J
Kanani
Amin
A
Stark
Donald
D
McCusker
Christine
C
Wagner
Eric
E
Rivard
Georges-Etienne
GE
Leith
Eric
E
Tsai
Ellie
E
MacSween
Michael
M
Lyanga
John
J
Serushago
Bazir
B
Leznoff
Art
A
Waserman
Susan
S
de Serres
Jean
J
D000465:Algorithms; D000799:Angioedema / Q000175:diagnosis* / Q000628:therapy*; D002170:Canada; D005260:Female; D006801:Humans; D007391:International Cooperation