Hereditary and acquired angioedema: Problems and progress: Proceedings of the third C1 esterase inhibitor deficiency workshop and beyond

Angelo Agostoni, Emel Aygören-Pürsün, Karen E. Binkley, Alvaro Blanch, Konrad Bork, Laurence Bouillet, Christoph Bucher, Anthony J. Castaldo, Marco Cicardi, Alvin E. Davis, Caterina De Carolis, Christian Drouet, Christiane Duponchel, Henriette Farkas, Kálmán Fáy, Béla Fekete, Bettina Fischer, Luigi Fontana, George Füst, Roberto GiacomelliAlbrecht Gröner, C. Erik Hack, George Harmat, John Jakenfelds, Mathias Juers, Lajos Kalmár, Pál N. Kaposi, István Karádi, Arianna Kitzinger, Tímea Kollár, Wolfhart Kreuz, Peter Lakatos, Hilary J. Longhurst, Margarita Lopez-Trascasa, Inmaculada Martinez-Saguer, Nicole Monnier, István Nagy, Éva Németh, Erik Waage Nielsen, Jan H. Nuijens, Caroline O'Grady, Emanuela Pappalardo, Vincenzo Penna, Carlo Perricone, Roberto Perricone, Ursula Rauch, Olga Roche, Eva Rusicke, Peter J. Späth, George Szendei, Edit Takács, Attila Tordai, Lennart Truedsson, Lilian Varga, Beáta Visy, Kayla Williams, Andrea Zanichelli, Lorenza Zingale

Research output: Contribution to journalArticle

473 Citations (Scopus)


Hereditary angioedema (HAE), a rare but life-threatening condition, manifests as acute attacks of facial, laryngeal, genital, or peripheral swelling or abdominal pain secondary to intra-abdominal edema. Resulting from mutations affecting C1 esterase inhibitor (C1-INH), inhibitor of the first complement system component, attacks are not histamine-mediated and do not respond to antihistamines or corticosteroids. Low awareness and resemblance to other disorders often delay diagnosis; despite availability of C1-INH replacement in some countries, no approved, safe acute attack therapy exists in the United States. The biennial C1 Esterase Inhibitor Deficiency Workshops resulted from a European initiative for better knowledge and treatment of HAE and related diseases. This supplement contains work presented at the third workshop and expanded content toward a definitive picture of angioedema in the absence of allergy. Most notably, it includes cumulative genetic investigations; multinational laboratory diagnosis recommendations; current pathogenesis hypotheses; suggested prophylaxis and acute attack treatment, including home treatment; future treatment options; and analysis of patient subpopulations, including pediatric patients and patients whose angioedema worsened during pregnancy or hormone administration. Causes and management of acquired angioedema and a new type of angioedema with normal C1-INH are also discussed. Collaborative patient and physician efforts, crucial in rare diseases, are emphasized. This supplement seeks to raise awareness and aid diagnosis of HAE, optimize treatment for all patients, and provide a platform for further research in this rare, partially understood disorder.

Original languageEnglish
Pages (from-to)S51-S131
JournalJournal of Allergy and Clinical Immunology
Issue number3 SUPPL.
Publication statusPublished - Sep 2004


  • AAE
  • C1 esterase inhibitor
  • C1-INH
  • HAE
  • HANE
  • HANO
  • acquired angioedema
  • angioedema
  • angioneurotic edema
  • chemically induced angioedema
  • hereditary angioedema
  • hereditary angioneurotic edema
  • human SERPING1 protein

ASJC Scopus subject areas

  • Immunology and Allergy
  • Immunology

Fingerprint Dive into the research topics of 'Hereditary and acquired angioedema: Problems and progress: Proceedings of the third C1 esterase inhibitor deficiency workshop and beyond'. Together they form a unique fingerprint.

  • Cite this

    Agostoni, A., Aygören-Pürsün, E., Binkley, K. E., Blanch, A., Bork, K., Bouillet, L., Bucher, C., Castaldo, A. J., Cicardi, M., Davis, A. E., De Carolis, C., Drouet, C., Duponchel, C., Farkas, H., Fáy, K., Fekete, B., Fischer, B., Fontana, L., Füst, G., ... Zingale, L. (2004). Hereditary and acquired angioedema: Problems and progress: Proceedings of the third C1 esterase inhibitor deficiency workshop and beyond. Journal of Allergy and Clinical Immunology, 114(3 SUPPL.), S51-S131.