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2010 International consensus algorithm for the diagnosis, therapy and management of hereditary angioedema
Tom Bowen1, Marco Cicardi, Henriette Farkas
1Departments of Medicine and Paediatrics, University of Calgary, Calgary, Alberta, Canada. tbowen@pol.net.
Insights
This study updates the 2010 International Consensus Algorithm for diagnosing and managing Hereditary Angioedema (HAE). The consensus provides an interim guide for this complex disorder, emphasizing the need for evidence-based guidelines from clinical trials.
Area of Science:
- Immunology
- Genetics
- Clinical Medicine
Background:
- Previous consensus algorithms for Hereditary Angioedema (HAE) were published in 2003 and updated in 2007.
- HAE is characterized by C1 inhibitor (C1-INH) deficiency.
Purpose of the Study:
- To update the International Consensus Algorithm for the Diagnosis, Therapy, and Management of Hereditary Angioedema (HAE) as of 2010.
Main Methods:
- A conference was held in Toronto, Canada, on May 15-16, 2010, by the Canadian Hereditary Angioedema Network (CHAEN) and cosponsors.
- The consensus document was reviewed at the conference and subsequently circulated for further review.
Main Results:
- The manuscript presents the 2010 International Consensus Algorithm for the Diagnosis, Therapy, and Management of Hereditary Angioedema.
Conclusions:
- The current consensus approach serves as an interim guide for managing HAE.
- The need for large-scale clinical trials (Phase III/IV), meta-analyses, registry validation, and head-to-head trials is highlighted.
- Development of evidence-based guidelines and standards for HAE management is crucial.
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.
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