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Management of Adult Patients With Drug Reaction With Eosinophilia and Systemic Symptoms: A Delphi-Based International Consensus

MC. Brüggen, S. Walsh, MM. Ameri, N. Anasiewicz, E. Maverakis, LE. French, S. Ingen-Housz-Oro, DRESS Delphi consensus group, R. Abe, M. Ardern-Jones, H. Assier, A. Barbaud, B. Bensaid, W. Bernal, C. Bernier, A. Brassard, E. Brezinová, R. Cabañas,...

. 2024 ; 160 (1) : 37-44. [pub] 20240101

Language English Country United States

Document type Journal Article

IMPORTANCE: Drug reaction with eosinophilia and systemic symptoms (DRESS) is a rare but potentially fatal drug hypersensitivity reaction. To our knowledge, there is no international consensus on its severity assessment and treatment. OBJECTIVE: To reach an international, Delphi-based multinational expert consensus on the diagnostic workup, severity assessment, and treatment of patients with DRESS. DESIGN, SETTING, AND PARTICIPANTS: The Delphi method was used to assess 100 statements related to baseline workup, evaluation of severity, acute phase, and postacute management of DRESS. Fifty-seven international experts in DRESS were invited, and 54 participated in the survey, which took place from July to September 2022. MAIN OUTCOMES/MEASURES: The degree of agreement was calculated with the RAND-UCLA Appropriateness Method. Consensus was defined as a statement with a median appropriateness value of 7 or higher (appropriate) and a disagreement index of lower than 1. RESULTS: In the first Delphi round, consensus was reached on 82 statements. Thirteen statements were revised and assessed in a second round. A consensus was reached for 93 statements overall. The experts agreed on a set of basic diagnostic workup procedures as well as severity- and organ-specific further investigations. They reached a consensus on severity assessment (mild, moderate, and severe) based on the extent of liver, kidney, and blood involvement and the damage of other organs. The panel agreed on the main lines of DRESS management according to these severity grades. General recommendations were generated on the postacute phase follow-up of patients with DRESS and the allergological workup. CONCLUSIONS AND RELEVANCE: This Delphi exercise represents, to our knowledge, the first international expert consensus on diagnostic workup, severity assessment, and management of DRESS. This should support clinicians in the diagnosis and management of DRESS and constitute the basis for development of future guidelines.

Center for Biomedical Research Network on Rare Diseases Madrid Spain

Center for Blistering Diseases Department of Dermatology University Medical Center Groningen University of Groningen Groningen the Netherlands

Center of Rare Diseases IDI IRCCS Rome Italy

Christine Kühne Center for Allergy Research and Education Davos Switzerland

CHU Lille Department of Dermatology Univ Lille INSERM U1286 Lille Inflammation Translational Research Institute F 59000 Lille France

Department of Allergy La Paz University Hospital IdiPAZ Madrid Spain

Department of Dermatology 2 Colentina Clinical Hospital Carol Davila University of Medicine and Pharmacy Bucharest Romania

Department of Dermatology and Allergology Tenon Hospital Sorbonne Universite Paris France

Department of Dermatology and Allergology University Hospital Salzburg of the Paracelsus Medical University Salzburg Austria

Department of Dermatology and Allergy Center Odense University Hospital University of Southern Denmark Odense Denmark

Department of Dermatology and Allergy Hannover Medical School Hannover Germany

Department of Dermatology and Allergy University Hospital Ludwig Maximilians University Munich Munich Germany

Department of Dermatology Bichat Claude Bernard Hospital Paris Diderot University Paris France

Department of Dermatology Brigham and Women's Hospital Harvard Medical School Boston Massachusetts

Department of Dermatology Clinical Experimental Sciences Faculty of Medicine University of Southampton Southampton England United Kingdom

Department of Dermatology Emek Medical Center Afula Israel

Department of Dermatology Henri Mondor University Hospital University of Paris Est Créteil Créteil France

Department of Dermatology Keck School of Medicine University of Southern California Los Angeles

Department of Dermatology King's College Hospital London England United Kingdom

Department of Dermatology Kyorin University School of Medicine Mitaka Japan

Department of Dermatology Lyon Natecia Hospital Lyon France

Department of Dermatology Miller School of Medicine University of Miami Miami Florida

Department of Dermatology Nara Medical University Kashihara Japan

Department of Dermatology National Taiwan University Hospital and National Taiwan University College of Medicine Taipei Taiwan

Department of Dermatology Ospedale San Bortolo Vicenza Italy

Department of Dermatology Perelman School of Medicine University of Pennsylvania Philadelphia

Department of Dermatology Singapore General Hospital Singapore Singapore

Department of Dermatology The Ohio State University Columbus Ohio

Department of Dermatology University Hospital Lübeck Lübeck Germany

Department of Dermatology University Hospital Nantes Nantes France

Department of Dermatology University Hospital of Bordeaux Bordeaux France

Department of Dermatology University Hospital of Zurich Zurich Switzerland

Department of Dermatology University of California Davis Sacramento California

Department of Dermatology University of California San Francisco

Department of Dermatology University of Debrecen Debrecen Hungary

Department of Dermatology University of Kansas Kansas City

Department of Dermatovenereology St Anne's Faculty Hospital Faculty of Medicine Masaryk University Brno Czech Republic

Department of Medicine and Pharmacology Vanderbilt University Medical Center Nashville Tennessee

Department of Oral Medicine Guy's and St Thomas' NHS Foundation Trust London England United Kingdom

Department of Paediatric Dermatology Colentina University Hospital Carol Davila University of Medicine and Pharmacy Bucharest Romania

Department of Skin and Allergic Diseases University of Helsinki and Helsinki University Central Hospital Helsinki Finland

Department of Skin and Venereal Diseases Lithuanian University of Health Sciences Hospital of LUHS Kauno Klinikos Kaunas Lithuania

Division of Allergology and Clinical Immunology Department of Pneumology and Allergology Inselspital Bern University Hospital University of Bern Bern Switzerland

Division of Dermatology Niigata University Graduate School of Medical and Dental Sciences Niigata Japan

Division of Dermatology Sunnybrook Health Sciences Centre Toronto Ontario Canada

Dr Phillip Frost Department of Dermatology and Cutaneous Surgery Miller School of Medicine University of Miami Miami Florida

Editor JAMA Dermatology

Faculty of Medicine University of Zurich Zurich Switzerland

Images in Dermatology Editor JAMA Dermatology

Liver Intensive Therapy Unit King's Critical Care King's College Hospital London England United Kingdom

PIELenRed Consortium Madrid Spain

Regional Pharmacovigilance Centre Pitié Salpêtrière Hospital APHP Sorbonne Université Paris France

Ruth and Bruce Rappaport Faculty of Medicine Technion Israel Institute of Technology Haifa Israel

Sorbonne Université INSERM Institut Pierre Louis d'Epidemiologie et de Sante Publique AP HP Sorbonne Universite Tenon Hospital Department of Dermatology and Allergology Paris France

Toxic Bullous Dermatoses TOXIBUL Reference Center Filière FIMARAD AP HP Henri Mondor Hospital Créteil France

ToxiTEN group European Reference Network for Rare Skin Diseases Paris France

Univ Paris Est Créteil EpiDermE Créteil France

References provided by Crossref.org

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$a Management of Adult Patients With Drug Reaction With Eosinophilia and Systemic Symptoms: A Delphi-Based International Consensus / $c MC. Brüggen, S. Walsh, MM. Ameri, N. Anasiewicz, E. Maverakis, LE. French, S. Ingen-Housz-Oro, DRESS Delphi consensus group, R. Abe, M. Ardern-Jones, H. Assier, A. Barbaud, B. Bensaid, W. Bernal, C. Bernier, A. Brassard, E. Brezinová, R. Cabañas, A. Cardones, CY. Chu, SL. Chua, V. Descamps, B. Didona, SJ. Divito, R. Dodiuk-Gad, S. Elman, K. Gaspar, CG. Mortz, N. Hama, HY. Lee, B. Horváth, L. Jörg, BH. Kaffenberger, V. Kucinskiene, B. Lebrun-Vignes, RJ. Lehloenya, D. Meyersburg, R. Micheletti, B. Milpied, F. Miyagawa, A. Mostaghimi, M. Nägeli, L. Naldi, E. Oppel, EJ. Phillips, T. Pirani, A. Ranki, T. Mälkönen, M. Rosenbach, C. Salavastru, D. Staumont-Salle, H. Sandberg, J. Setterfield, K. Shinkai, T. Shiohara, A. Soria, D. Tartar, GS. Tiplica, S. Traidl, A. Vorobyev, C. von Wachter, S. Worswick, YT. Cho
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$a IMPORTANCE: Drug reaction with eosinophilia and systemic symptoms (DRESS) is a rare but potentially fatal drug hypersensitivity reaction. To our knowledge, there is no international consensus on its severity assessment and treatment. OBJECTIVE: To reach an international, Delphi-based multinational expert consensus on the diagnostic workup, severity assessment, and treatment of patients with DRESS. DESIGN, SETTING, AND PARTICIPANTS: The Delphi method was used to assess 100 statements related to baseline workup, evaluation of severity, acute phase, and postacute management of DRESS. Fifty-seven international experts in DRESS were invited, and 54 participated in the survey, which took place from July to September 2022. MAIN OUTCOMES/MEASURES: The degree of agreement was calculated with the RAND-UCLA Appropriateness Method. Consensus was defined as a statement with a median appropriateness value of 7 or higher (appropriate) and a disagreement index of lower than 1. RESULTS: In the first Delphi round, consensus was reached on 82 statements. Thirteen statements were revised and assessed in a second round. A consensus was reached for 93 statements overall. The experts agreed on a set of basic diagnostic workup procedures as well as severity- and organ-specific further investigations. They reached a consensus on severity assessment (mild, moderate, and severe) based on the extent of liver, kidney, and blood involvement and the damage of other organs. The panel agreed on the main lines of DRESS management according to these severity grades. General recommendations were generated on the postacute phase follow-up of patients with DRESS and the allergological workup. CONCLUSIONS AND RELEVANCE: This Delphi exercise represents, to our knowledge, the first international expert consensus on diagnostic workup, severity assessment, and management of DRESS. This should support clinicians in the diagnosis and management of DRESS and constitute the basis for development of future guidelines.
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$a DRESS Delphi consensus group
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