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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Graphical abstract

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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

Methods

Literature search

DANCE steering committee and expert panel

Development of rationale and concept of DANCE

Establishment of consensus by Delphi process

Results

Definition of AE and related terms

AE due to intrinsic vascular endothelium dysfunction

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.