2019 European League Against Rheumatism/American College of Rheumatology classification criteria for systemic lupus erythematosus
ObjectiveTo 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).MethodsThis international initiative had four phases. (1) Evaluation of antinuclear antibody (ANA)...
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Published in | Annals of the rheumatic diseases Vol. 78; no. 9; pp. 1151 - 1159 |
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Main Authors | , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , |
Format | Journal Article |
Language | English |
Published |
United States
BMJ Publishing Group Ltd and European League Against Rheumatism
01.09.2019
Elsevier Limited |
Subjects | |
Online Access | Get full text |
ISSN | 0003-4967 1468-2060 1468-2060 |
DOI | 10.1136/annrheumdis-2018-214819 |
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Abstract | ObjectiveTo 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).MethodsThis 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 1001 subjects and validation compared with previous criteria in a new validation cohort of 1270 subjects.ResultsThe 2019 EULAR/ACR classification criteria for SLE include positive ANA at least once as obligatory entry criterion; followed by additive weighted criteria grouped in seven clinical (constitutional, haematological, neuropsychiatric, mucocutaneous, serosal, musculoskeletal, renal) and three immunological (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.ConclusionThese 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 reflect current thinking about SLE and provide an improved foundation for SLE research. |
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AbstractList | 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).OBJECTIVETo 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 1001 subjects and validation compared with previous criteria in a new validation cohort of 1270 subjects.METHODSThis 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 1001 subjects and validation compared with previous criteria in a new validation cohort of 1270 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 seven clinical (constitutional, haematological, neuropsychiatric, mucocutaneous, serosal, musculoskeletal, renal) and three immunological (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.RESULTSThe 2019 EULAR/ACR classification criteria for SLE include positive ANA at least once as obligatory entry criterion; followed by additive weighted criteria grouped in seven clinical (constitutional, haematological, neuropsychiatric, mucocutaneous, serosal, musculoskeletal, renal) and three immunological (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 reflect current thinking about SLE and provide an improved foundation for SLE research.CONCLUSIONThese 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 reflect current thinking about SLE and provide an improved foundation for SLE research. ObjectiveTo 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).MethodsThis 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 1001 subjects and validation compared with previous criteria in a new validation cohort of 1270 subjects.ResultsThe 2019 EULAR/ACR classification criteria for SLE include positive ANA at least once as obligatory entry criterion; followed by additive weighted criteria grouped in seven clinical (constitutional, haematological, neuropsychiatric, mucocutaneous, serosal, musculoskeletal, renal) and three immunological (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.ConclusionThese 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 reflect current thinking about SLE and provide an improved foundation for SLE research. 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 1001 subjects and validation compared with previous criteria in a new validation cohort of 1270 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 seven clinical (constitutional, haematological, neuropsychiatric, mucocutaneous, serosal, musculoskeletal, renal) and three immunological (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 reflect current thinking about SLE and provide an improved foundation for SLE research. |
Author | Jacobsen, Søren Bertsias, George Touma, Zahi Tani, Chiara Diamond, Betty Meroni, Pier Luigi Jayne, David Sanchez-Guerrero, Jorge Pego-Reigosa, José M Boumpas, Dimitrios T Smolen, Josef S Tedeschi, Sara K Schmajuk, Gabriela Johnson, Sindhu R Hahn, Bevra Aringer, Martin Wofsy, David Padjen, Ivan Yavuz, Sule Gladman, Dafna D Costenbader, Karen McCune, Joseph Halda-Kiss, Bernadett Wallace, Daniel J Hoyer, Bimba F Ruiz-Irastorza, Guillermo Massarotti, Elena Schneider, Matthias Anic, Branimir Doria, Andrea Naden, Ray Kamen, Diane L Costedoat-Chalumeau, Nathalie Brinks, Ralph Lerstrøm, Kirsten Mosca, Marta Ramsey-Goldman, Rosalind Khanna, Dinesh Rúa-Figueroa Fernández, Íñigo Hasni, Sarfaraz Vital, Edward M Cervera, Ricard Tanaka, Yoshiya Daikh, David Seror, Raphaèle Kumánovics, Gábor Stummvoll, Georg H Dörner, Thomas Graninger, Winfried Izmirly, Peter M Assan, Florence Czirják, László Mariette, Xavier Vasconcelos, Carlos Jung, Michelle Hiepe, Falk Clarke, Ann Elaine Urowitz, Murray Chan, Tak Mao Tektonidou, Maria G Leuchten, Nicolai Romero-Diaz, Juanit |
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Medicine, Azienda Ospedaliero Universitaria Pisana, University of Pisa, Pisa, Italy – sequence: 6 givenname: Rosalind surname: Ramsey-Goldman fullname: Ramsey-Goldman, Rosalind organization: Department of Medicine, Division of Rheumatology, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA – sequence: 7 givenname: Josef S surname: Smolen fullname: Smolen, Josef S organization: Department of Rheumatology, Medicine III, Medical University of Vienna, Vienna, Austria – sequence: 8 givenname: David surname: Wofsy fullname: Wofsy, David organization: Department of Medicine, Russell/Engleman Rheumatology Research Center, University of California at San Francisco, San Francisco, California, USA – sequence: 9 givenname: Dimitrios T surname: Boumpas fullname: Boumpas, Dimitrios T organization: Departments of Internal Medicine and Rheumatology, Clinical Immunology and Allergy, Medical School, University of Cyprus, Nicosia, Cyprus – sequence: 10 givenname: Diane L surname: Kamen fullname: Kamen, Diane L organization: Department of Medicine, Medical University of South Carolina, Charleston, South Carolina, USA – sequence: 11 givenname: David surname: Jayne fullname: Jayne, David organization: Department of Medicine, University of Cambridge, Cambridge, UK – sequence: 12 givenname: Ricard surname: Cervera fullname: Cervera, Ricard organization: Department of Autoimmune Diseases, Hospital Clínic, University of Barcelona, Barcelona, Spain – sequence: 13 givenname: Nathalie orcidid: 0000-0002-1555-9021 surname: Costedoat-Chalumeau fullname: Costedoat-Chalumeau, Nathalie organization: Medicine, Toronto Western Hospital, University Health Network, Mount Sinai Hospital, University of Toronto, Toronto Scleroderma Research Program, Toronto, Ontario, Canada – sequence: 14 givenname: Betty orcidid: 0000-0002-3250-3804 surname: Diamond fullname: Diamond, Betty organization: Center for Autoimmune, Musculoskeletal and Hematopoietic Diseases, The Feinstein 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Hoyer, Bimba F organization: Department of Medicine III, University of Schleswig-Holstein at Kiel, Kiel, Germany – sequence: 29 givenname: Nicolai surname: Leuchten fullname: Leuchten, Nicolai organization: Medicine III, University Medical Center and Faculty of Medicine Carl Gustav Carus, TU Dresden, Dresden, Germany – sequence: 30 givenname: Chiara surname: Tani fullname: Tani, Chiara organization: Department of Clinical and Experimental Medicine, Rheumatology Unit, Azienda Ospedaliero Universitaria Pisana, University of Pisa, Pisa, Italy – sequence: 31 givenname: Sara K orcidid: 0000-0001-9475-1363 surname: Tedeschi fullname: Tedeschi, Sara K organization: Department of Medicine, Brigham and Women's Hospital, Boston, Massachusetts, USA – sequence: 32 givenname: Zahi surname: Touma fullname: Touma, Zahi organization: Division of Rheumatology, Department of Medicine, Toronto Western Hospital, Institute of Health Policy, Management and Evaluation, University of Toronto, Toronto, 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University of Calgary, Calgary, Alberta, Canada – sequence: 38 givenname: Mary K surname: Crow fullname: Crow, Mary K organization: Hospital for Special Surgery, Ney York, New York, USA – sequence: 39 givenname: László surname: Czirják fullname: Czirják, László organization: Department of Rheumatology and Immunology, University of Pécs, Pécs, Hungary – sequence: 40 givenname: Andrea orcidid: 0000-0003-0548-4983 surname: Doria fullname: Doria, Andrea organization: Rheumatology Unit, Department of Medicine (DIMED), University of Padova, Padova, Italy – sequence: 41 givenname: Winfried surname: Graninger fullname: Graninger, Winfried organization: Internal Medicine, Medical University of Graz, Graz, Austria – sequence: 42 givenname: Bernadett surname: Halda-Kiss fullname: Halda-Kiss, Bernadett organization: Department of Rheumatology and Immunology, University of Pécs, Pécs, Hungary – sequence: 43 givenname: Sarfaraz surname: Hasni fullname: Hasni, Sarfaraz organization: Lupus Clinical 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Université Paris-Saclay, Le Kremlin Bicêtre, France – sequence: 48 givenname: Ivan surname: Padjen fullname: Padjen, Ivan organization: Division of Clinical Immunology and Rheumatology, University of Zagreb School of Medicine and University Hospital Centre Zagreb, Zagreb, Croatia – sequence: 49 givenname: José M surname: Pego-Reigosa fullname: Pego-Reigosa, José M organization: Department of Rheumatology, University Hospital of Vigo, IRIDIS Group, Instituto de Investigación Sanitaria Galicia Sur (IISGS), Vigo, Spain – sequence: 50 givenname: Juanita surname: Romero-Diaz fullname: Romero-Diaz, Juanita organization: Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico – sequence: 51 givenname: Íñigo surname: Rúa-Figueroa Fernández fullname: Rúa-Figueroa Fernández, Íñigo organization: Rheumatology, Doctor Negrín University Hospital, Las Palmas de Gran Canaria, Las Palmas, Spain – sequence: 52 givenname: Raphaèle surname: Seror fullname: Seror, Raphaèle organization: Department of Rheumatology, Université Paris Sud, Hôpitaux Universitaires Paris-Sud, AP-HP, INSERM UMR 1184, Le Kremlin-Bicêtre, France – sequence: 53 givenname: Georg H surname: Stummvoll fullname: Stummvoll, Georg H organization: Rheumatology, Medical University of Vienna, Vienna, Austria – sequence: 54 givenname: Yoshiya orcidid: 0000-0002-0807-7139 surname: Tanaka fullname: Tanaka, Yoshiya organization: First Department of Internal Medicine, University of Occupational and Environmental Health, Japan, Kitakyushu, Japan – sequence: 55 givenname: Maria G orcidid: 0000-0003-2238-0975 surname: Tektonidou fullname: Tektonidou, Maria G organization: Medical School, National and Kapodistrian University of Athens, Athens, Greece – sequence: 56 givenname: Carlos surname: Vasconcelos fullname: Vasconcelos, Carlos organization: Clinical Immunology Unit, Centro Hospitalar do Porto, ICBAS, University of Porto, Porto, Portugal – sequence: 57 givenname: Edward M surname: Vital fullname: Vital, Edward M organization: NIHR Leeds Biomedical Research Centre, Leeds Teaching Hospitals NHS Trust, Leeds, UK – sequence: 58 givenname: Daniel J surname: Wallace fullname: Wallace, Daniel J organization: Division of Rheumatology, Cedars-Sinai Medical Center, Los Angeles, California, USA – sequence: 59 givenname: Sule surname: Yavuz fullname: Yavuz, Sule organization: Department of Rheumatology, Istanbul Bilim University, Istanbul, Turkey – sequence: 60 givenname: Pier Luigi surname: Meroni fullname: Meroni, Pier Luigi organization: Clinical Immunology and Rheumatology Unit, IRCCS Istituto Auxologico Italiano, Milan, Italy – sequence: 61 givenname: Marvin J surname: Fritzler fullname: Fritzler, Marvin J organization: Faculty of Medicine, Cumming School of Medicine, University of Calgary, Calgary, Alberta, Canada – sequence: 62 givenname: Ray surname: Naden fullname: Naden, Ray organization: Maternal-Fetal Medicine, McMaster University Faculty of Health Sciences, Hamilton, Ontario, Canada – sequence: 63 givenname: Thomas orcidid: 0000-0002-6478-7725 surname: Dörner fullname: Dörner, Thomas organization: Department of Rheumatology and Clinical Immunology, Charité – Universitätsmedizin Berlin, Corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Berlin, Germany – sequence: 64 givenname: Sindhu R surname: Johnson fullname: Johnson, Sindhu R email: Sindhu.Johnson@uhn.ca organization: Institute of Health Policy, Management and Evaluation, University of Toronto, Toronto, Ontario, Canada |
BackLink | https://www.ncbi.nlm.nih.gov/pubmed/31383717$$D View this record in MEDLINE/PubMed |
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Snippet | ObjectiveTo develop new classification criteria for systemic lupus erythematosus (SLE) jointly supported by the European League Against Rheumatism (EULAR) and... To develop new classification criteria for systemic lupus erythematosus (SLE) jointly supported by the European League Against Rheumatism (EULAR) and the... |
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SubjectTerms | Antibodies Antinuclear antibodies Antiphospholipid antibodies Arthritis Classification classification criteria consensus methods Criteria Humans Immunoglobulins Immunology Literature reviews Lupus Lupus Erythematosus, Systemic - classification Medical research multi-criteria decision analysis Pathogenesis Patients Rheumatic Diseases Rheumatism Rheumatology Societies, Medical Systemic lupus erythematosus validation |
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Title | 2019 European League Against Rheumatism/American College of Rheumatology classification criteria for systemic lupus erythematosus |
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