Review
2010 International consensus algorithm for the diagnosis, therapy and management of hereditary angioedema
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* Corresponding author: Tom Bowen tbowen@pol.net
1 Departments of Medicine and Paediatrics, University of Calgary, Calgary, Alberta, Canada
2 Department of Internal Medicine, Universita degli Studi di Milano, Ospedale L. Sacco, Milan, Italy
3 3rd Department of Internal Medicine, Faculty of Medicine, Semmelweis University, Budapest, Hungary
4 Department of Dermatology, University Hospital of the Johannes Gutenberg-University of Mainz, Mainz, Germany
5 Department of Immunology, Barts and the London NHS Trust, London, England, UK
6 University of California, San Diego, San Diego, California, USA
7 Johann Wolfgang Goethe University, Frankfurt/Main, Germany
8 Departments of Medicine and Pediatrics, Penn State University, Hershey, Pennsylvania, USA
9 Department of Medicine, University of Toronto, Toronto, Canada
10 Department of Medicine, Laval University, Quebec City, Quebec, Canada
11 Departments of Medicine and Medical Oncology, University of Alberta, Edmonton, Alberta, Canada
12 Department of Medicine, CHU de Grenoble, Grenoble, France
13 Member, Patient Advisory Committee, Canadian Hereditary Angioedema Network (CHAEN)/Réseau Canadien d'angioédème héréditaire (RCAH). 705 South Tower, 3031 Hospital Dr. NW, Calgary, Alberta, Canada
14 Portage La Prairie, Manitoba, Canada
15 Department of Pediatrics, University of British Columbia, Vancouver, British Columbia, Canada
16 Department of Medicine, Regina, Saskatchewan, Canada
17 Memorial University and Janeway Child Health Centre, St. John's, Newfoundland, Canada
18 Department of Medicine, McMaster University, Hamilton, Ontario, Canada
19 Department of Medicine, Dalhousie University, Halifax, Nova Scotia, Canada
20 Department of Medicine, University of Toronto, Oakville, Ontario, Canada
21 Ancaster, Ontario, Canada
22 St. Catharines, Ontario, Canada; Member and Chair, Patient Advisory Committee, Canadian Hereditary Angioedema Network (CHAEN)/Réseau Canadien d'angioédème héréditaire (RCAH
23 Transfusion Medicine, Ottawa Hospital, Ottawa, Ontario, Canada
24 Department of Medicine, University of Calgary, Calgary, Alberta, Canada
25 Department of Pediatrics, CHU Sainte-Justine, University of Montreal, Montreal, Quebec, Canada
26 Department of Medicine, University of British Columbia, Vancouver, British Columbia, Canada
27 Halifax, Nova Scotia, Canada
28 Brampton, Ontario, Canada
29 Queen's University, Kingston, Ontario, Canada
30 Department of Medicine, University of Manitoba, Winnipeg, Manitoba, Canada
31 University of Auckland, Auckland, New Zealand
32 Department of Internal Medicine, University of Cincinnati, Cincinnati, Ohio, USA
33 Department of Clinical and Experimental Medicine, County Hospital Ryhov, Jönköping, Sweden
34 Calgary, Alberta, Canada
35 Department of Dermatology and Allergy Centre, Odense University Hospital, Denmark
36 Hospital La Paz Health Research Institute, Madrid, Spain
37 Duke University Medical Center, Durham, North Carolina, USA
38 Heim Pal Pediatric Hospital, Budapest, Hungary
39 Dept of Medicine, Academic Medical Center, Amsterdam Area, Netherlands
40 Institute for Asthma & Allergy, Wheaton and Chevy Chase, Maryland, USA
41 4th Medical Clinic, University of Medicine and Pharmacy, Tirgu Mures, Romania
42 Hungarian Association of Angioedema Patients, Budapest, Hungary
43 Nordland Hospital, Bodo, University of Tromso, Norway
44 Department of Medicine, County Hospital Ryhov, Jonkoping, Sweden
45 Tel Hashomer, and Sackler Faculty of Medicine, Tel Aviv University, Ramat Aviv, Israel
46 Asociación Española de Angioedema Familiar por Deficiencia del inhibidor de C1 (AEDAF), Madrid, Spain
47 Institute of Pharmacology, University of Bern, Switzerland
48 Peking Union Medical College Hospital, Beijing, China
Allergy, Asthma & Clinical Immunology 2010, 6:24 doi:10.1186/1710-1492-6-24
Published: 28 July 2010Abstract
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 webcite 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.