Advanced

Hereditary and acquired angioedema: problems and progress: proceedings of the third C1 esterase inhibitor deficiency workshop and beyond

Agostoni, Angelo; Aygoren-Pursun, Emel; Binkley, Karen E; Blanch, Alvaro; Bork, Konrad; Bouillet, Laurence; Bucher, Christoph; Castaldo, Anthony J; Cicardi, Marco and Davis, Alvin E, et al. (2004) In Journal of Allergy and Clinical Immunology 114(3 Suppl). p.51-131
Abstract
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... (More)
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. (Less)
Please use this url to cite or link to this publication:
author
, et al. (More)
(Less)
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
HANO, hereditary angioedema, HANE, HAE, C1-INH, angioedema, AAE, C1 esterase inhibitor, hereditary angioneurotic edema, angioneurotic edema, chemically induced angioedema, human SERPING1 protein, acquired angioedema
in
Journal of Allergy and Clinical Immunology
volume
114
issue
3 Suppl
pages
51 - 131
publisher
Elsevier
external identifiers
  • pmid:15356535
  • scopus:4444225379
ISSN
1097-6825
DOI
10.1016/j.jaci.2004.06.047
language
English
LU publication?
yes
id
92d6df0a-ea17-4a15-99f3-f6a463bb54dc (old id 1129677)
date added to LUP
2008-06-12 11:24:25
date last changed
2017-12-10 04:36:24
@article{92d6df0a-ea17-4a15-99f3-f6a463bb54dc,
  abstract     = {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.},
  author       = {Agostoni, Angelo and Aygoren-Pursun, Emel and Binkley, Karen E and Blanch, Alvaro and Bork, Konrad and Bouillet, Laurence and Bucher, Christoph and Castaldo, Anthony J and Cicardi, Marco and Davis, Alvin E and De Carolis, Caterina and Drouet, Christian and Duponchel, Christiane and Farkas, Hen and Fáy, Kálmán and Fekete, Béla and Fischer, Bettina and Fontana, Luigi and Füst, George and Giacomelli, Roberto and Gröner, Albrecht and Hack, Erik and Harmat, George and Jakenfelds, John and Juers, Mathias and Kalmár, Lajos and Kaposi, Pál N. and Karádi, István and Kitzinger, Arianna and Kollár, Tímea and Kreuz, Wolfhart and Lakatos, Peter and Longhurst, Hilary J. and Lopez-Trascasa, Margarita and Martinez-Saguer, Inmaculada and Monnier, Nicole and Nagy, István and Németh, Éva and Waage Nielsen, Erik and Nuijens, Jan H. and O'Grady, Caroline and Pappalardo, Emanuela and Penna, Vincenzo and Perricone, Carlo and Perricone, Roberto and Rauch, Ursula and Roche, Olga and Rusicke, Eva and Späth, Peter J and Szendei, George and Takács, Edit and Tordai, Attila and Truedsson, Lennart and Varga, Lilian and Visy, Beáta and Williams, Kayla and Zanichelli, Andrea and Zingale, Lorenza},
  issn         = {1097-6825},
  keyword      = {HANO,hereditary angioedema,HANE,HAE,C1-INH,angioedema,AAE,C1 esterase inhibitor,hereditary angioneurotic edema,angioneurotic edema,chemically induced angioedema,human SERPING1 protein,acquired angioedema},
  language     = {eng},
  number       = {3 Suppl},
  pages        = {51--131},
  publisher    = {Elsevier},
  series       = {Journal of Allergy and Clinical Immunology},
  title        = {Hereditary and acquired angioedema: problems and progress: proceedings of the third C1 esterase inhibitor deficiency workshop and beyond},
  url          = {http://dx.doi.org/10.1016/j.jaci.2004.06.047},
  volume       = {114},
  year         = {2004},
}