Evidence-based recommendations for the therapeutic management of angioedema owing to hereditary C1 inhibitor deficiency: Consensus report of an International Working Group

Marco Cicardi, Konrad Bork, Teresa Caballero, Timothy Craig, H. H. Li, Hilary Longhurst, Avner Reshef, Bruce Zuraw, Werner Aberer, Emel Aygören-Pürsün, Aleena Banerji, Janne Bjorkander, Isabelle Boccon-Gibod, Laurence Bouillet, France Grenoble, Maria Bova, Tom Bowen, Canada Calgary, Ferreira Manuel Branco, Anette BygumTeresa Caballero, Mauro Cancian, Maria Graça Castel-Branco, Caterina de Carolis, Enikö Mihály, Josè Fabiani, Henriette Farkas, Mark Gompels, Richard Gower, Adriane Groffik, Anete Grumach, Mar Guillarte, Erik Hack, Landeros Maria Hernandez, Allen Kaplan, Alicia Lara, Iris Leibovich, Henry Li, Bob Lock, William Lumry, Alejandro Malbran, Inmaculada Martinez-Saguer, Campos Matta, Marcus Maurer, Dumitru Moldovan, Vincenzo Montinaro, Sandra Nieto, Patrik Nordenfelt, Krystyna Obtulovicz, Roberto Perricone, Nieves Prior, Marc Riedl, Valle Rodrigues do, Chiara Savoca, Peter Spaeth, Petra Staubach-Renz, Marcin Stobiecki, Massimo Triggiani, Romualdo Vacchini, Lilian Varga, Andrea Zanichelli, Kian Zarchi, Sacha Zeerleder, Lorenza Zingale

Research output: Contribution to journalReview article

Abstract

A ngioedema owing to hereditary deficiency of C1 inhibitor (HAE) is a rare, life-threatening, disabling disease. In the last 2 years, the results of well-designed and controlled trials with existing and new therapies for this condition have been published, and new treatments reached the market. Current guidelines for the treatment for HAE were released before the new trials and before the new treatments became available and were essentially based on observational studies and expert opinion. To provide evidence-based HAE treatment guidelines supported by the new studies, a conference was held in Gargnano del Garda, Italy, from September 26 to 29, 2010. The meeting hosted 58 experienced HAE expert physicians, representatives of pharmaceutical companies and representatives of HAE patients' associations. Here, we report the topics discussed during the meeting and evidence-based consensus about management approaches for HAE in adult/adolescent patients.

Original languageEnglish (US)
Pages (from-to)147-157
Number of pages11
JournalAllergy: European Journal of Allergy and Clinical Immunology
Volume67
Issue number2
DOIs
StatePublished - Feb 1 2012

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Keywords

  • C1 inhibitor
  • bradykinin receptor antagonist
  • consensus document
  • hereditary angioedema
  • kallikrein inhibitor

ASJC Scopus subject areas

  • Immunology and Allergy
  • Immunology

Cite this

Cicardi, M., Bork, K., Caballero, T., Craig, T., Li, H. H., Longhurst, H., Reshef, A., Zuraw, B., Aberer, W., Aygören-Pürsün, E., Banerji, A., Bjorkander, J., Boccon-Gibod, I., Bouillet, L., Grenoble, F., Bova, M., Bowen, T., Calgary, C., Branco, F. M., ... Zingale, L. (2012). Evidence-based recommendations for the therapeutic management of angioedema owing to hereditary C1 inhibitor deficiency: Consensus report of an International Working Group. Allergy: European Journal of Allergy and Clinical Immunology, 67(2), 147-157. https://doi.org/10.1111/j.1398-9995.2011.02751.x