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Definition, acronyms, nomenclature, and classification of angioedema (DANCE): AAAAI, ACAAI, ACARE, and APAAACI DANCE consensus - 17/05/24

Doi : 10.1016/j.jaci.2024.03.024 
Avner Reshef, MD a, , Thomas Buttgereit, MD b, c, Stephen D. Betschel, MD d, Teresa Caballero, MD, PhD e, mm, Henriette Farkas, MD, PhD, DSc f, Anete S. Grumach, MD, PhD g, Michihiro Hide, MD, PhD h, Ankur K. Jindal, MD i, Hilary Longhurst, MD j, Jonathan Peter, PhD k, Marc A. Riedl, MD l, Yuxiang Zhi, MD m, Werner Aberer, MD n, Mohamed Abuzakouk, PhD o, Tariq Al Farsi, MD p, Nashat Al Sukaiti, MD p, Mona Al-Ahmad, MD q, Sabine Altrichter, MD r, Emel Aygören-Pürsün, MD s, Maria Luisa Baeza, MD, PhD t, Noemi Anna Bara, MD u, Andrea Bauer, MD v, Jonathan A. Bernstein, MD w, Isabelle Boccon-Gibod, MD x, Hanna Bonnekoh, MD b, c, Laurence Bouillet, MD, PhD x, y, Zenon Brzoza, MD, PhD z, Anette Bygum, MD, DMSc aa, bb, Oscar Calderon, MD cc, Regis de Albuquerque Campos, MD, PhD dd, Freya Helena Campos Romero, MD ee, Mauro Cancian, MD, PhD ff, Herberto Jose Chong-Neto, MD, PhD gg, George Christoff, MD, PhD hh, ii, Stefan Cimbollek, MD jj, Danny M. Cohn, MD, PhD kk, Timothy Craig, DO ll, Inna Danilycheva, MD mm, Razvigor Darlenski, MD, PhD nn, Aurélie Du-Thanh, MD, PhD oo, Luis Felipe Ensina, MD, PhD pp, Daria Fomina, MD qq, Luz Fonacier, MD rr, Atsushi Fukunaga, MD, PhD ss, Asli Gelincik, MD tt, Pedro Giavina-Bianchi, MD, PhD uu, Kiran Godse, MD, PhD vv, Mark Gompels, MD ww, Margarida Goncalo, MD, PhD xx, Maia Gotua, MD, PhD yy, Guillermo Guidos-Fogelbach, MD, MsC, PhDc zz, Mar Guilarte, MD, PhD aaa, Alicja Kasperska-Zajac, MD, PhD bbb, Constance H. Katelaris, MD, PhD ccc, Tamar Kinaciyan, MD ddd, Pavel Kolkhir, MD b, c, Kanokvalai Kulthanan, MD eee, Marcin Kurowski, MD, PhD fff, Elena Latysheva, MD, PhD mm, ggg, Antti Lauerma, MD, PhD hhh, David Launay, MD, PhD iii, Ramon Lleonart, MD jjj, William Lumry, MD kkk, Alejandro Malbran, MD, PhD lll, Ramzy Mohammed Ali, MD mmm, Iman Nasr, MD nnn, Sandra Nieto-Martinez, MD, PhD ooo, Claudio Parisi, MD ppp, Ruby Pawankar, MD, PhD qqq, Macarena Piñero-Saavedra, MD rrr, Todor A. Popov, MD, PhD sss, Grzegorz Porebski, MD ttt, Alicia Prieto Garcia, MD, PhD uuu, Polina Pyatilova, MD b, c, Michael Rudenko, MD, PhD vvv, Bulent Enis Sekerel, MD www, Faradiba Sarquis Serpa, PhD xxx, Farrukh Sheikh, MD yyy, Frank Siebenhaar, MD b, c, Angèle Soria, MD, PhD zzz, Maria Staevska, MD, PhD aaaa, Petra Staubach, MD bbbb, Marcin Stobiecki, MD, PhD ttt, Simon Francis Thomsen, MD, PhD cccc, Massimo Triggiani, MD, PhD dddd, Anna Valerieva, MD, PhD aaaa, Solange Valle, MD, PhD eeee, Nguyen Van Dinh, MD, PhD ffff, Carolina Elisa Vera Ayala, MD b, c, Anna Zalewska-Janowska, MD, PhD gggg, Andrea Zanichelli, MD, PhD hhhh, iiii, Markus Magerl, MD b, c, Marcus Maurer, MD b, c
a Angioedema Research Center, Barzilai University Medical Center, Ashkelon, Israel 
b Angioedema Center of Reference and Excellence (ACARE), Institute of Allergology, Charité–Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany 
c Fraunhofer Institute for Translational Medicine, and Pharmacology ITMP, Immunology and Allergology, Berlin, Germany 
d Department of Medicine, St Michael’s Hospital, University of Toronto, Toronto, Ontario, Canada 
e Department of Allergy, La Paz University Hospital, Hospital La Paz Institute for Health Research (IdiPAZ—Group 44), Biomedical Research Network on Rare Diseases (CIBERER U754), Madrid, Spain 
f Hungarian Angioedema Center of Reference and Excellence, Department of Internal Medicine and Haematology, Semmelweis University, Budapest, Hungary 
g Department of Clinical Immunology, University Center Faculdade de Medicina do ABC, Santo André, Brazil 
h Department of Dermatology, Institute of Biomedical & Health Sciences, Hiroshima University, Hiroshima, Japan 
i Department of Pediatrics, Allergy Immunology Unit, Postgraduate Institute of Medical Education and Research, Chandigarh, India 
j Department of Medicine, University of Auckland and Department of Immunology, Auckland City Hospital, Auckland, New Zealand 
k Department of Medicine, Division of Allergy and Clinical Immunology, University of Cape Town, and the Allergy and Immunology Unit, University of Cape Town Lung Institute, Cape Town, South Africa 
l Division of Rheumatology, Allergy, and Immunology, University of California San Diego, La Jolla, Calif 
m Department of Allergy, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China 
n Department of Dermatology, Medical University of Graz, Graz, Austria 
o Allergy and Immunology, Respiratory Institute, Cleveland Clinic Abu Dhabi, Abu Dhabi, United Arab Emirates 
p Department of Pediatric Allergy and Clinical Immunology, The Royal Hospital, Muscat, Oman 
q Microbiology Department, College of Medicine, Kuwait University, Kuwait City, Kuwait 
r Klinik für Dermatologie und Venerologie, Kepler Uniklinikum, Linz, Austria 
s Department of Pediatrics, University Hospital Frankfurt, Goethe University, Frankfurt, Germany 
t Allergy Department, Hospital General Universitario Gregorio Marañón, Biomedical Research Network on Rare Diseases–U761, Gregorio Marañón Health Research Institute (IiSGM), Gregorio Marañón, Madrid, Spain 
u Romanian Hereditary Angioedema Expertise Centre, Centrul Clinic Mediquest, Sângeorgiu de Mure, Romania 
v Department of Dermatology, University Hospital Carl Gustav Carus, Technical University Dresden, Dresden, Germany 
w University of Cincinnati College of Medicine, Department of Internal Medicine, Division of Rheumatology, Allergy and Immunology, Cincinnati, Ohio 
x National Reference Center of Angioedema CREAK, Grenoble, France 
y Internal medicine department, Grenoble University Hospital, Grenoble, France 
z Department of Internal Diseases with Division of Allergology, Institute of Medical Sciences, University of Opole, Opole, Poland 
aa Department of Clinical Genetics, Odense University Hospital, Odense, Denmark 
bb Clinical Institute, University of Southern Denmark, Odense, Denmark 
cc Clínica SANNA el Golf, San Isidro, Lima, Peru 
dd Universidade Federal da Bahia, Brazilian Network of Hereditary Angioedema, Salvador, Brazil 
ee Department of Allergy and Clinical Immunology, Hospital Central Sur Alta Especialidad, Mexico City, Mexico City, Mexico 
ff Departmental Unit of Allergology, University Hospital of Padua, Padua, Italy 
gg Serviço de Alergia e Imunologia, Complexo Hospital de Clinicas, Universidade Federal do Paraná, Curitiba, Brazil 
hh Excelsior Medical Centre, Sofia, Bulgaria 
ii Medical University–Sofia, Sofia, Bulgaria 
jj Hospital Universitario Virgen del Rocío, Seville, Spain 
kk Department of Vascular Medicine, Amsterdam UMC, Amsterdam Cardiovascular Sciences, University of Amsterdam, Amsterdam, The Netherlands 
ll Department of Pediatrics, Pennsylvania State University, Hershey, Pa 
mm NRC Institute of Immunology FMBA of Russia, Moscow, Russia 
nn Department of Dermatovenereology, Trakia University, Stara Zagora, Bulgaria 
oo Département de dermatologie, ACARE, Centre Hospitalier Universitaire de Montpellier Montpellier, France 
pp Federal University of São Paulo, São Paulo, Brazil 
qq Moscow City Research and Practical Center of Allergoloy and Immunology, Clinical Hospital No. 52, Moscow Healthcare Department, Moscow, Russia 
rr New York University–Long Island School of Medicine, Mineola, NY 
ss Department of Dermatology, Division of Medicine for Function and Morphology of Sensory Organs, Faculty of Medicine, Osaka Medical and Pharmaceutical University, Osaka, Japan 
tt Division of Immunology and Allergic Diseases, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey 
uu Division of Clinical lmmunology and Allergy, Universidade de São Paulo, São Paulo, São Paulo, Brazil 
vv Dr D. Y. Patil Medical College and Hospital, Navi Mumbai, Maharashtra, India 
ww Department of Immunology, ACARE, North Bristol NHS Trust, Bristol, United Kingdom 
xx Department of Dermatology, Coimbra Hospital and University Center, and Faculty of Medicine, University of Coimbra, Coimbra, Portugal 
yy Center of Allergy and Immunology and David Tvildiani Medical University, Tbilisi, Georgia 
zz SEPI/ENMH Instituto Politecnico Nacional, Mexico City, Mexico 
aaa Allergy Department, Hospital Universitari Vall d’Hebron, Vall d’Hebron Research Institute (VHIR), Barcelona, Spain 
bbb European Center for Diagnosis and Treatment of Urticaria and Angioedema and Department of Clinical Allergology and Urticaria, Medical University of Silesia in Katowice, Katowice, Poland 
ccc Western Sydney University and Campbelltown Hospital, Campbelltown, Australia 
ddd Department of Dermatology, Medical University of Vienna, Vienna, Austria 
eee Department of Dermatology, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand 
fff Department of Immunology and Allergy, Medical University of Lodz, Lodz, Poland 
ggg Pirogov Russian National Research Medical University, Moscow, Russia 
hhh Department of Dermatology, Skin and Allergy Hospital, Helsinki University Hospital, Helsinki, Finland 
iii University Lille, Inserm, CHU Lille, Service de Médecine Interne et Immunologie Clinique, Centre de Référence des Angioedemes à Kinine (CREAK), U1286-INFINITE–Institute for Translational Research in Inflammation, Lille, France 
jjj Allergology Department, Hospital Universitari Bellvitge, IDIBILL Research Institute, L’Hospitalet de Llobregat, Barcelona, Spain 
kkk AARA Research Center, Dallas, Tex 
lll Unidad de Alergia, Asma e Inmunología Clínica, Buenos Aires, Argentina 
mmm Department of Medicine, Allergy and Immunology Division, Hamad Medical Corporation, Doha, Qatar 
nnn Immunology and Allergy Department, The Royal Hospital, Muscat, Oman 
ooo Unidad de Genética de la Nutrición, Instituto Nacional de Pediatría, Mexico City, Mexico 
ppp Pediatric and Adult Allergy sections of the Italian Hospital of Buenos Aires, Buenos Aires, Argentina 
qqq Nippon Medical School, Tokyo, Japan 
rrr Allergy Department, Centro Hospitalar do Algarve, Faro, Portugal 
sss University Hospital Sv Ivan Rilski, Sofia, Bulgaria 
ttt Department of Clinical and Environmental Allergology, Jagiellonian University Medical College, Krakow, Poland 
uuu Department of Allergy, Gregorio Marañón Health Research Institute (IiSGM), Gregorio Marañón University Hospital, Madrid, Spain 
vvv The London Allergy and Immunology Centre, London, United Kingdom 
www Pediatric Allergy and Asthma Division, Hacettepe University, Ankara, Turkey 
xxx Angioedema and Urticaria Reference Center, Hospital Santa Casa de Misericórdia de Vitória, Vitória, Espirito Santo, Brazil 
yyy Chughtai Medical Center, Lahore, Pakistan 
zzz Médecine Sorbonne Université, Service de Dermatologie et Allergologie, hôpital Tenon, Assistance Publique Hôpitaux de Paris, Paris, France 
aaaa Department of Allergology, Medical University of Sofia, Clinic of Allergology, University Hospital “Alexandrovska” Sofia, Bulgaria 
bbbb Department of Dermatology, ACARE, University Medical Center Mainz, Mainz, Germany 
cccc Department of Dermatovenereology, Bispebjerg Hospital, Copenhagen, Denmark 
dddd Division of Allergy and Clinical Immunology, University of Salerno, Salerno, Italy 
eeee Department of Internal Medicine, Immunology Service, Hospital Universitario Clementino Fraga Filho, Rio De Janiero, Brazil 
ffff Department of General Internal Medicine, Respiratory–Allergy and Clinical Immunology Unit, Vinmec Healthcare System, Hanoi, Vietnam 
gggg Department of Psychodermatology, Medical University of Lodz, Lodz, Poland 
hhhh Department of Biomedical Sciences for Health, Università degli Studi di Milano, Milan, Italy 
iiii Operative Unit of Medicine, IRCCS Policlinico San Donato, San Donato Milanese, Milan, Italy 

Corresponding author: Avner Reshef, MD, Angioedema Research Center, Barzilai University Medical Center, Ashkelon, Israel.Angioedema Research CenterBarzilai University Medical CenterAshkelonIsrael
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Abstract

Background

Angioedema (AE) manifests with intermittent, localized, self-limiting swelling of the subcutaneous and/or submucosal tissue. AE is heterogeneous, can be hereditary or acquired, may occur only once or be recurrent, may exhibit wheals or not, and may be due to mast cell mediators, bradykinin, or other mechanisms. Several different taxonomic systems are currently used, making it difficult to compare the results of studies, develop multicenter collaboration, and harmonize AE treatment.

Objective

We developed a consensus on the definition, acronyms, nomenclature, and classification of AE (DANCE).

Methods

The initiative involved 91 experts from 35 countries and was endorsed by 53 scientific and medical societies, and patient organizations. A consensus was reached by online discussion and voting using the Delphi process over a period of 16 months (June 2021 to November 2022).

Results

The DANCE initiative resulted in an international consensus on the definition, classification, and terminology of AE. The new consensus classification features 5 types and endotypes of AE and a harmonized vocabulary of abbreviations/acronyms.

Conclusion

The DANCE classification complements current clinical guidelines and expert consensus recommendations on the diagnostic assessment and treatment of AE. DANCE does not replace current clinical guidelines, and expert consensus algorithms and should not be misconstrued in a way that affects reimbursement of medicines prescribed by physicians using sound clinical judgment. We anticipate that this new AE taxonomy and nomenclature will harmonize and facilitate AE research and clinical studies, thereby improving patient care.

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Key words : Angioedema, acronyms, classification, terminology, types


Plan


 The first 2 authors contributed equally to this article, and both should be considered first author. The last 2 authors contributed equally to this article, and both should be considered senior author.


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