Performance of the 2019 EULAR/ACR classification criteria for systemic lupus erythematosus in early disease, across sexes and ethnicities

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

doi:10.1136/annrheumdis-2020-217162

Performance of the 2019 EULAR/ACR classification criteria for systemic lupus erythematosus in early disease, across sexes and ethnicities

Ann Rheum Dis. 2020 Oct;79(10):1333-1339. doi: 10.1136/annrheumdis-2020-217162. Epub 2020 Aug 14.

Authors

Sindhu R Johnson¹, Ralph Brinks², Karen H Costenbader^{3

4}, David Daikh⁵, Marta Mosca⁶, Rosalind Ramsey-Goldman⁷, Josef S Smolen⁸, David Wofsy⁹, Dimitrios T Boumpas^{10

11}, Diane L Kamen¹², David Jayne¹³, R Cervera¹⁴, Nathalie Costedoat-Chalumeau^{15

16}, Betty Diamond¹⁷, Dafna D Gladman^{18

19}, Bevra Hahn²⁰, Falk Hiepe²¹, Søren Jacobsen²², Dinesh Khanna²³, Kirsten Lerstrøm²⁴, Elena Massarotti^{4

25}, Joseph McCune²³, Guillermo Ruiz-Irastorza²⁶, Jorge Sanchez-Guerrero^{27

28}, Matthias Schneider²⁹, Murray Urowitz³⁰, George Bertsias^{31

32}, Bimba F Hoyer³³, Nicolai Leuchten³⁴, Chiara Tani^{35

36}, Sara K Tedeschi^{4

37}, Zahi Touma³⁸, Gabriela Schmajuk³⁹, Branimir Anic⁴⁰, Florence Assan⁴¹, Tak Mao Chan⁴², Ann Elaine Clarke⁴³, Mary K Crow⁴⁴, László Czirják⁴⁵, Andrea Doria⁴⁶, Winfried B Graninger⁴⁷, Bernadett Halda-Kiss⁴⁵, Sarfaraz Hasni⁴⁸, Peter M Izmirly⁴⁹, Michelle Jung⁵⁰, Gábor Kumánovics⁴⁵, Xavier Mariette⁵¹, Ivan Padjen⁵², José M Pego-Reigosa⁵³, Juanita Romero-Diaz⁵⁴, Íñigo Rúa-Figueroa⁵⁵, Raphaèle Seror⁴¹, Georg H Stummvoll⁸, Yoshiya Tanaka⁵⁶, Maria G Tektonidou⁵⁷, Carlos Vasconcelos⁵⁸, Edward M Vital^{59

60}, D J Wallace⁶¹, Sule Yavuz⁶², Pier Luigi Meroni⁶³, Marvin J Fritzler⁶⁴, Ray Naden⁶⁵, Thomas Dörner²¹, Martin Aringer⁶⁶

Affiliations

¹ Division of Rheumatology, Department of Medicine, Toronto Western Hospital, Mount Sinai Hospital; Institute of Health Policy, Management and Evaulation, University of Toronto, Toronto, Ontario, Canada [email protected] [email protected].
² Policlinic and Hiller Research Unit of Rheumatology, Heinrich-Heine-University Duesseldorf, Düsseldorf, Germany.
³ Department of Medicine, Division of Rheumatology, Inflammation, and Immunity, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts, USA.
⁴ Rheumatology, Harvard Medical School, Boston, Massachusetts, USA.
⁵ Medicine/Rheumatology, UCSF Medical Center, San Francisco, California, USA.
⁶ Rheumatology Unit, University of Pisa, Pisa, Italy.
⁷ Medicine/Rheumatology, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA.
⁸ Rheumatology, Medical University of Vienna, Vienna, Austria.
⁹ Division of Rheumatology, , Russell/Engleman Rheumatology Research Center, University of California, San Francisco, San Francisco, California, USA.
¹⁰ Medicine, National and Kapodestrian University of Athens, and Biomedical Research Foundation of the Athens Academy, Athens, Greece.
¹¹ Rheumatology, University of Cyprus Medical School, Nicosia, Nicosia, Cyprus.
¹² Rheumatology, Medical University of South Carolina, Charleston, South Carolina, USA.
¹³ Medicine, University of Cambridge, Cambridge, UK.
¹⁴ Autoimmune Diseases, Hospital Clinic, University of Barcelona, Barcelona, Catalonia, Spain.
¹⁵ Internal Medicine, Hospital Cochin, Paris, Île-de-France, France.
¹⁶ INSERM U 1153, Center for Epidemiology and Statistics Sorbonne Paris Cité, Université Paris Descartes-Sorbonne Paris Cité, Paris, France.
¹⁷ Autoimmune and Musculoskeletal, Feinstein Institutes for Medical Research, Manhasset, New York, USA.
¹⁸ Medicine, University of Toronto, Toronto, Ontario, Canada.
¹⁹ Medicine, Toronto Western Hospital, Toronto, Ontario, Canada.
²⁰ Rheumatology, UCLA School of Medicine, Los Angeles, California, USA.
²¹ Rheumatology and Clinical Immunology, Charité-Universitätsmedizin Berlin, Corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Berlin, Germany.
²² Department of Rheumatology, Copenhagen Lupus and Vasculitis Clinic, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark.
²³ Division of Rheumatology, University of Michigan, Ann Arbor, Michigan, USA.
²⁴ Lupus Europe, Romford, UK.
²⁵ Medicine, Brigham and Women's Hospital, Boston, Massachusetts, USA.
²⁶ Autoimmune Diseases Unit, Biocruces Bizkaia Health Research Institute, Hospital Universitario Cruces, Barakaldo, País Vasco, Spain.
²⁷ Medicine, Mount Sinai Hospital/University Health Network, University of Toronto, Toronto, Ontario, Canada.
²⁸ Immunology and Rheumatology, Instituto Nacional de Ciencias Medicas y Nutricion, Mexico City, Mexico.
²⁹ Policlinic and Hiller Research Unit for Rheumatology, Medical Faculty,Heinrich-Heine-University, Düsseldorf, Germany.
³⁰ Medicine, Toronto Western Hospital, University of Toronto, Toronto, Ontario, Canada.
³¹ Rheumatology, University of Crete School of Medicine, Iraklio, Crete, Greece.
³² Laboratory of Autoimmunity-Inflammation, Institute of Molecular Biology and Biotechnology, Heraklion, Crete, Greece.
³³ Rheumatology and Clinical Immunology, University of Schleswig-Holstein at Kiel, Kiel, Germany.
³⁴ Medicine III, University Medical Center and Faculty of Medicine Carl Gustav Carus, TU Dresden, Dresden, Germany.
³⁵ Rheumatology, Azienda Ospedaliero Universitaria Pisana, Pisa, Toscana, Italy.
³⁶ Rheumatology, University of Pisa, Pisa, Toscana, Italy.
³⁷ Medicine, Division of Rheumatology, Brigham and Women's Hospital, Boston, Massachusetts, USA.
³⁸ Division of Rheumatology, Department of Medicine, Toronto Western Hospital, Institute of Health Policy, Management and Evaluation, University of Toronto, Toronto, Ontario, Canada.
³⁹ Department of Medicine, Division of Rheumatology, University of California, San Francisco, San Francisco, California, USA.
⁴⁰ Division of Clinical Immunology and Rheumatology, Department of Medicine, University Hospital Centre Zagreb, Zagreb, Croatia.
⁴¹ INSERM UMR 1163, Université Paris Sud, Hôpitaux Universitaires Paris-Sud, AP-HP, INSERM UMR 1184, Paris, France.
⁴² Department of Medicine, University of Hong Kong, Pokfulam, Hong Kong.
⁴³ Rheumatology, University of Calgary Cumming School of Medicine, Calgary, Alberta, Canada.
⁴⁴ Mary Kirkland Center for Lupus Research, Hospital for Special Surgery, New York, New York, USA.
⁴⁵ Department of Rheumatology and Immunology, University of Pécs, Pécs, Hungary.
⁴⁶ Division of Rheumatology, University of Padova, Padova, Italy.
⁴⁷ Rheumatology and Immunology, Medical University Graz, Graz, Austria.
⁴⁸ Rheumatology, NIAMS, National Institutes of Health, Bethesda, Maryland, USA.
⁴⁹ Rheumatology, New York University School of Medicine, New York, New York, USA.
⁵⁰ Rheumatology, University of Calgary, Calgary, Alberta, Canada.
⁵¹ Hôpitaux Universitaires Paris-Sud, AP-HP, INSERM UMR, Université Paris Sud, Le Kremlin-Bicêtre, France.
⁵² Division of Clinical Immunology and Rheumatology, University of Zagreb School of Medicine and University Hospital Centre Zagreb, Zagreb, Croatia.
⁵³ Rheumatology, University Hospital of Vigo, IRIDIS Group, Instituto de Investigación Sanitaria Galicia Sur, Vigo, Spain.
⁵⁴ Immunology and Rheumatology, Instituto Nacional de Ciencias Medicas y Nutricion Salvador Zubiran, Tlalpan, Mexico.
⁵⁵ Rheumatology, Dr Negrin University Hospital of Gran Canaria, Las Palmas de Gran Canaria, Spain.
⁵⁶ First Department of Internal Medicine, University of Occupational and Environmental Health, Japan, Kitakyushu, Japan.
⁵⁷ Medical School, National and Kapodistrian University of Athens, Athinon, Greece.
⁵⁸ Centro Hospitalar do Porto, ICBAS, University of Porto, Porto, Portugal.
⁵⁹ Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK.
⁶⁰ NIHR Leeds Biomedical Research Centre, Leeds Teaching Hospitals NHS Trust, Leeds, UK.
⁶¹ Rheumatology, Cedars-Sinai Medical Center, Los Angeles, California, USA.
⁶² Rheumatology, Istanbul Bilim Universitesi, Istanbul, Turkey.
⁶³ Clinical Immunology and Rheumatology, IRCCS Istituto Auxologico Italiano, Milan, Italy.
⁶⁴ Medicine, University of Calgary Cumming School of Medicine, Calgary, Alberta, Canada.
⁶⁵ Medicine, McMaster University Faculty of Health Sciences, Hamilton, Ontario, Canada.
⁶⁶ Rheumatology, University Medical Center and Faculty of Medicine Carl Gustav Carus, Dresden, Germany [email protected] [email protected].

PMID: 32816709
DOI: 10.1136/annrheumdis-2020-217162

Abstract

Objectives: The European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) 2019 Classification Criteria for systemic lupus erythematosus (SLE) have been validated with high sensitivity and specificity. We evaluated the performance of the new criteria with regard to disease duration, sex and race/ethnicity, and compared its performance against the Systemic Lupus International Collaborating Clinics (SLICC) 2012 and ACR 1982/1997 criteria.

Methods: Twenty-one SLE centres from 16 countries submitted SLE cases and mimicking controls to form the validation cohort. The sensitivity and specificity of the EULAR/ACR 2019, SLICC 2012 and ACR 1982/1997 criteria were evaluated.

Results: The cohort consisted of female (n=1098), male (n=172), Asian (n=118), black (n=68), Hispanic (n=124) and white (n=941) patients; with an SLE duration of 1 to <3 years (n=196) and ≥5 years (n=879). Among patients with 1 to <3 years disease duration, the EULAR/ACR criteria had better sensitivity than the ACR criteria (97% vs 81%). The EULAR/ACR criteria performed well in men (sensitivity 93%, specificity 96%) and women (sensitivity 97%, specificity 94%). Among women, the EULAR/ACR criteria had better sensitivity than the ACR criteria (97% vs 83%) and better specificity than the SLICC criteria (94% vs 82%). Among white patients, the EULAR/ACR criteria had better sensitivity than the ACR criteria (95% vs 83%) and better specificity than the SLICC criteria (94% vs 83%). The EULAR/ACR criteria performed well among black patients (sensitivity of 98%, specificity 100%), and had better sensitivity than the ACR criteria among Hispanic patients (100% vs 86%) and Asian patients (97% vs 77%).

Conclusions: The EULAR/ACR 2019 criteria perform well among patients with early disease, men, women, white, black, Hispanic and Asian patients. These criteria have superior sensitivity than the ACR criteria and/or superior specificity than the SLICC criteria across many subgroups.

Keywords: epidemiology; lupus nephritis; outcomes research; systemic lupus erythematosus.

Publication types

Research Support, N.I.H., Intramural
Research Support, Non-U.S. Gov't

MeSH terms

Female
Humans
Lupus Erythematosus, Systemic / classification*
Male
Patient Selection
Sensitivity and Specificity
Severity of Illness Index*

Abstract

Publication types

MeSH terms

Grants and funding