2019 European League Against Rheumatism/American College of Rheumatology Classification Criteria for Systemic Lupus Erythematosus. 2019

Martin Aringer, and Karen Costenbader, and David Daikh, and Ralph Brinks, and Marta Mosca, and Rosalind Ramsey-Goldman, and Josef S Smolen, and David Wofsy, and Dimitrios T Boumpas, and Diane L Kamen, and David Jayne, and Ricard Cervera, and Nathalie Costedoat-Chalumeau, and Betty Diamond, and Dafna D Gladman, and Bevra Hahn, and Falk Hiepe, and Søren Jacobsen, and Dinesh Khanna, and Kirsten Lerstrøm, and Elena Massarotti, and Joseph McCune, and Guillermo Ruiz-Irastorza, and Jorge Sanchez-Guerrero, and Matthias Schneider, and Murray Urowitz, and George Bertsias, and Bimba F Hoyer, and Nicolai Leuchten, and Chiara Tani, and Sara K Tedeschi, and Zahi Touma, and Gabriela Schmajuk, and Branimir Anic, and Florence Assan, and Tak Mao Chan, and Ann Elaine Clarke, and Mary K Crow, and László Czirják, and Andrea Doria, and Winfried Graninger, and Bernadett Halda-Kiss, and Sarfaraz Hasni, and Peter M Izmirly, and Michelle Jung, and Gábor Kumánovics, and Xavier Mariette, and Ivan Padjen, and José M Pego-Reigosa, and Juanita Romero-Diaz, and Íñigo Rúa-Figueroa Fernández, and Raphaèle Seror, and Georg H Stummvoll, and Yoshiya Tanaka, and Maria G Tektonidou, and Carlos Vasconcelos, and Edward M Vital, and Daniel J Wallace, and Sule Yavuz, and Pier Luigi Meroni, and Marvin J Fritzler, and Ray Naden, and Thomas Dörner, and Sindhu R Johnson
University Medical Center and Faculty of Medicine Carl Gustav Carus, TU Dresden, Dresden, Germany.

To develop new classification criteria for systemic lupus erythematosus (SLE) jointly supported by the European League Against Rheumatism (EULAR) and the American College of Rheumatology (ACR). This international initiative had four phases. 1) Evaluation of antinuclear antibody (ANA) as an entry criterion through systematic review and meta-regression of the literature and criteria generation through an international Delphi exercise, an early patient cohort, and a patient survey. 2) Criteria reduction by Delphi and nominal group technique exercises. 3) Criteria definition and weighting based on criterion performance and on results of a multi-criteria decision analysis. 4) Refinement of weights and threshold scores in a new derivation cohort of 1,001 subjects and validation compared with previous criteria in a new validation cohort of 1,270 subjects. The 2019 EULAR/ACR classification criteria for SLE include positive ANA at least once as obligatory entry criterion; followed by additive weighted criteria grouped in 7 clinical (constitutional, hematologic, neuropsychiatric, mucocutaneous, serosal, musculoskeletal, renal) and 3 immunologic (antiphospholipid antibodies, complement proteins, SLE-specific antibodies) domains, and weighted from 2 to 10. Patients accumulating ≥10 points are classified. In the validation cohort, the new criteria had a sensitivity of 96.1% and specificity of 93.4%, compared with 82.8% sensitivity and 93.4% specificity of the ACR 1997 and 96.7% sensitivity and 83.7% specificity of the Systemic Lupus International Collaborating Clinics 2012 criteria. These new classification criteria were developed using rigorous methodology with multidisciplinary and international input, and have excellent sensitivity and specificity. Use of ANA entry criterion, hierarchically clustered, and weighted criteria reflects current thinking about SLE and provides an improved foundation for SLE research.

UI MeSH Term Description Entries
D007391 International Cooperation The interaction of persons or groups of persons representing various nations in the pursuit of a common goal or interest. Foreign Aid,Treaties,Aid, Foreign,Cooperation, International,Treaty
D008180 Lupus Erythematosus, Systemic A chronic, relapsing, inflammatory, and often febrile multisystemic disorder of connective tissue, characterized principally by involvement of the skin, joints, kidneys, and serosal membranes. It is of unknown etiology, but is thought to represent a failure of the regulatory mechanisms of the autoimmune system. The disease is marked by a wide range of system dysfunctions, an elevated erythrocyte sedimentation rate, and the formation of LE cells in the blood or bone marrow. Libman-Sacks Disease,Lupus Erythematosus Disseminatus,Systemic Lupus Erythematosus,Disease, Libman-Sacks,Libman Sacks Disease
D008297 Male Males
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D003165 Complement System Proteins Serum glycoproteins participating in the host defense mechanism of COMPLEMENT ACTIVATION that creates the COMPLEMENT MEMBRANE ATTACK COMPLEX. Included are glycoproteins in the various pathways of complement activation (CLASSICAL COMPLEMENT PATHWAY; ALTERNATIVE COMPLEMENT PATHWAY; and LECTIN COMPLEMENT PATHWAY). Complement Proteins,Complement,Complement Protein,Hemolytic Complement,Complement, Hemolytic,Protein, Complement,Proteins, Complement,Proteins, Complement System
D003661 Decision Support Techniques Mathematical or statistical procedures used as aids in making a decision. They are frequently used in medical decision-making. Decision Analysis,Decision Modeling,Models, Decision Support,Analysis, Decision,Decision Aids,Decision Support Technics,Aid, Decision,Aids, Decision,Analyses, Decision,Decision Aid,Decision Analyses,Decision Support Model,Decision Support Models,Decision Support Technic,Decision Support Technique,Model, Decision Support,Modeling, Decision,Technic, Decision Support,Technics, Decision Support,Technique, Decision Support,Techniques, Decision Support
D003697 Delphi Technique An iterative questionnaire designed to measure consensus among individual responses. In the classic Delphi approach, there is no interaction between responder and interviewer. Delphi Method,Delphi Studies,Delphi Technic,Delphi Methods,Delphi Study,Delphi Technics,Delphi Techniques,Method, Delphi,Methods, Delphi,Studies, Delphi,Study, Delphi,Technic, Delphi,Technics, Delphi,Technique, Delphi,Techniques, Delphi
D005060 Europe The continent north of AFRICA, west of ASIA and east of the ATLANTIC OCEAN. Northern Europe,Southern Europe,Western Europe
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man

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