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

Martin Aringer, Karen Costenbader, David Daikh, Ralph Brinks, Marta Mosca, Rosalind Ramsey-Goldman, Josef S. Smolen, David Wofsy, Dimitrios T. Boumpas, Diane L. Kamen, David Jayne, Ricard Cervera, Nathalie Costedoat-Chalumeau, Betty Diamond, Dafna D. Gladman, Bevra Hahn, Falk Hiepe, Søren Jacobsen, Dinesh Khanna, Kirsten LerstrømElena 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 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 Fernández, Raphaèle Seror, Georg H. Stummvoll, Yoshiya Tanaka, Maria G. Tektonidou, Carlos Vasconcelos, Edward M. Vital, Daniel J. Wallace, Sule Yavuz, Pier Luigi Meroni, Marvin J. Fritzler, Ray Naden, Thomas Dörner, Sindhu R. Johnson

Research output: Contribution to journalArticle

10 Citations (Scopus)

Abstract

Objective: 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). Methods: 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. Results: 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. Conclusion: 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.

Original languageEnglish
Pages (from-to)1400-1412
Number of pages13
JournalArthritis and Rheumatology
Volume71
Issue number9
DOIs
Publication statusPublished - Sep 1 2019

Fingerprint

Systemic Lupus Erythematosus
Antinuclear Antibodies
Sensitivity and Specificity
Rheumatology
Exercise
Antiphospholipid Antibodies
Decision Support Techniques
Rheumatic Diseases
Complement System Proteins
Kidney
Weights and Measures
Antibodies
Research

ASJC Scopus subject areas

  • Immunology and Allergy
  • Rheumatology
  • Immunology

Cite this

Aringer, M., Costenbader, K., Daikh, D., Brinks, R., Mosca, M., Ramsey-Goldman, R., ... Johnson, S. R. (2019). 2019 European League Against Rheumatism/American College of Rheumatology Classification Criteria for Systemic Lupus Erythematosus. Arthritis and Rheumatology, 71(9), 1400-1412. https://doi.org/10.1002/art.40930

2019 European League Against Rheumatism/American College of Rheumatology Classification Criteria for Systemic Lupus Erythematosus. / Aringer, Martin; Costenbader, Karen; Daikh, David; Brinks, Ralph; Mosca, Marta; Ramsey-Goldman, Rosalind; Smolen, Josef S.; Wofsy, David; Boumpas, Dimitrios T.; Kamen, Diane L.; Jayne, David; Cervera, Ricard; Costedoat-Chalumeau, Nathalie; Diamond, Betty; Gladman, Dafna D.; Hahn, Bevra; Hiepe, Falk; Jacobsen, Søren; Khanna, Dinesh; Lerstrøm, Kirsten; Massarotti, Elena; McCune, Joseph; Ruiz-Irastorza, Guillermo; Sanchez-Guerrero, Jorge; Schneider, Matthias; Urowitz, Murray; Bertsias, George; Hoyer, Bimba F.; Leuchten, Nicolai; Tani, Chiara; Tedeschi, Sara K.; Touma, Zahi; Schmajuk, Gabriela; Anic, Branimir; Assan, Florence; Chan, Tak Mao; Clarke, Ann Elaine; Crow, Mary K.; Czirják, László; Doria, Andrea; Graninger, Winfried; Halda-Kiss, Bernadett; Hasni, Sarfaraz; Izmirly, Peter M.; Jung, Michelle; Kumánovics, Gábor; Mariette, Xavier; Padjen, Ivan; Pego-Reigosa, José M.; Romero-Diaz, Juanita; Rúa-Figueroa Fernández, Íñigo; Seror, Raphaèle; Stummvoll, Georg H.; Tanaka, Yoshiya; Tektonidou, Maria G.; Vasconcelos, Carlos; Vital, Edward M.; Wallace, Daniel J.; Yavuz, Sule; Meroni, Pier Luigi; Fritzler, Marvin J.; Naden, Ray; Dörner, Thomas; Johnson, Sindhu R.

In: Arthritis and Rheumatology, Vol. 71, No. 9, 01.09.2019, p. 1400-1412.

Research output: Contribution to journalArticle

Aringer, M, Costenbader, K, Daikh, D, Brinks, R, Mosca, M, Ramsey-Goldman, R, Smolen, JS, Wofsy, D, Boumpas, DT, Kamen, DL, Jayne, D, Cervera, R, Costedoat-Chalumeau, N, Diamond, B, Gladman, DD, Hahn, B, Hiepe, F, Jacobsen, S, Khanna, D, Lerstrøm, K, Massarotti, E, McCune, J, Ruiz-Irastorza, G, Sanchez-Guerrero, J, Schneider, M, Urowitz, M, Bertsias, G, Hoyer, BF, Leuchten, N, Tani, C, Tedeschi, SK, Touma, Z, Schmajuk, G, Anic, B, Assan, F, Chan, TM, Clarke, AE, Crow, MK, Czirják, L, Doria, A, Graninger, W, Halda-Kiss, B, Hasni, S, Izmirly, PM, Jung, M, Kumánovics, G, Mariette, X, Padjen, I, Pego-Reigosa, JM, Romero-Diaz, J, Rúa-Figueroa Fernández, Í, Seror, R, Stummvoll, GH, Tanaka, Y, Tektonidou, MG, Vasconcelos, C, Vital, EM, Wallace, DJ, Yavuz, S, Meroni, PL, Fritzler, MJ, Naden, R, Dörner, T & Johnson, SR 2019, '2019 European League Against Rheumatism/American College of Rheumatology Classification Criteria for Systemic Lupus Erythematosus', Arthritis and Rheumatology, vol. 71, no. 9, pp. 1400-1412. https://doi.org/10.1002/art.40930
Aringer, Martin ; Costenbader, Karen ; Daikh, David ; Brinks, Ralph ; Mosca, Marta ; Ramsey-Goldman, Rosalind ; Smolen, Josef S. ; Wofsy, David ; Boumpas, Dimitrios T. ; Kamen, Diane L. ; Jayne, David ; Cervera, Ricard ; Costedoat-Chalumeau, Nathalie ; Diamond, Betty ; Gladman, Dafna D. ; Hahn, Bevra ; Hiepe, Falk ; Jacobsen, Søren ; Khanna, Dinesh ; Lerstrøm, Kirsten ; Massarotti, Elena ; McCune, Joseph ; Ruiz-Irastorza, Guillermo ; Sanchez-Guerrero, Jorge ; Schneider, Matthias ; Urowitz, Murray ; Bertsias, George ; Hoyer, Bimba F. ; Leuchten, Nicolai ; Tani, Chiara ; Tedeschi, Sara K. ; Touma, Zahi ; Schmajuk, Gabriela ; Anic, Branimir ; Assan, Florence ; Chan, Tak Mao ; Clarke, Ann Elaine ; Crow, Mary K. ; Czirják, László ; Doria, Andrea ; Graninger, Winfried ; Halda-Kiss, Bernadett ; Hasni, Sarfaraz ; Izmirly, Peter M. ; Jung, Michelle ; Kumánovics, Gábor ; Mariette, Xavier ; Padjen, Ivan ; Pego-Reigosa, José M. ; Romero-Diaz, Juanita ; Rúa-Figueroa Fernández, Íñigo ; Seror, Raphaèle ; Stummvoll, Georg H. ; Tanaka, Yoshiya ; Tektonidou, Maria G. ; Vasconcelos, Carlos ; Vital, Edward M. ; Wallace, Daniel J. ; Yavuz, Sule ; Meroni, Pier Luigi ; Fritzler, Marvin J. ; Naden, Ray ; Dörner, Thomas ; Johnson, Sindhu R. / 2019 European League Against Rheumatism/American College of Rheumatology Classification Criteria for Systemic Lupus Erythematosus. In: Arthritis and Rheumatology. 2019 ; Vol. 71, No. 9. pp. 1400-1412.
@article{f7af54f68ff945e983f73b8b2e8bafff,
title = "2019 European League Against Rheumatism/American College of Rheumatology Classification Criteria for Systemic Lupus Erythematosus",
abstract = "Objective: 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). Methods: 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. Results: 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. Conclusion: 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.",
author = "Martin Aringer and Karen Costenbader and David Daikh and Ralph Brinks and Marta Mosca and Rosalind Ramsey-Goldman and Smolen, {Josef S.} and David Wofsy and Boumpas, {Dimitrios T.} and Kamen, {Diane L.} and David Jayne and Ricard Cervera and Nathalie Costedoat-Chalumeau and Betty Diamond and Gladman, {Dafna D.} and Bevra Hahn and Falk Hiepe and S{\o}ren Jacobsen and Dinesh Khanna and Kirsten Lerstr{\o}m and Elena Massarotti and Joseph McCune and Guillermo Ruiz-Irastorza and Jorge Sanchez-Guerrero and Matthias Schneider and Murray Urowitz and George Bertsias and Hoyer, {Bimba F.} and Nicolai Leuchten and Chiara Tani and Tedeschi, {Sara K.} and Zahi Touma and Gabriela Schmajuk and Branimir Anic and Florence Assan and Chan, {Tak Mao} and Clarke, {Ann Elaine} and Crow, {Mary K.} and L{\'a}szl{\'o} Czirj{\'a}k and Andrea Doria and Winfried Graninger and Bernadett Halda-Kiss and Sarfaraz Hasni and Izmirly, {Peter M.} and Michelle Jung and G{\'a}bor Kum{\'a}novics and Xavier Mariette and Ivan Padjen and Pego-Reigosa, {Jos{\'e} M.} and Juanita Romero-Diaz and {R{\'u}a-Figueroa Fern{\'a}ndez}, {\'I}{\~n}igo and Rapha{\`e}le Seror and Stummvoll, {Georg H.} and Yoshiya Tanaka and Tektonidou, {Maria G.} and Carlos Vasconcelos and Vital, {Edward M.} and Wallace, {Daniel J.} and Sule Yavuz and Meroni, {Pier Luigi} and Fritzler, {Marvin J.} and Ray Naden and Thomas D{\"o}rner and Johnson, {Sindhu R.}",
year = "2019",
month = "9",
day = "1",
doi = "10.1002/art.40930",
language = "English",
volume = "71",
pages = "1400--1412",
journal = "Arthritis and Rheumatology",
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TY - JOUR

T1 - 2019 European League Against Rheumatism/American College of Rheumatology Classification Criteria for Systemic Lupus Erythematosus

AU - Aringer, Martin

AU - Costenbader, Karen

AU - Daikh, David

AU - Brinks, Ralph

AU - Mosca, Marta

AU - Ramsey-Goldman, Rosalind

AU - Smolen, Josef S.

AU - Wofsy, David

AU - Boumpas, Dimitrios T.

AU - Kamen, Diane L.

AU - Jayne, David

AU - Cervera, Ricard

AU - Costedoat-Chalumeau, Nathalie

AU - Diamond, Betty

AU - Gladman, Dafna D.

AU - Hahn, Bevra

AU - Hiepe, Falk

AU - Jacobsen, Søren

AU - Khanna, Dinesh

AU - Lerstrøm, Kirsten

AU - Massarotti, Elena

AU - McCune, Joseph

AU - Ruiz-Irastorza, Guillermo

AU - Sanchez-Guerrero, Jorge

AU - Schneider, Matthias

AU - Urowitz, Murray

AU - Bertsias, George

AU - Hoyer, Bimba F.

AU - Leuchten, Nicolai

AU - Tani, Chiara

AU - Tedeschi, Sara K.

AU - Touma, Zahi

AU - Schmajuk, Gabriela

AU - Anic, Branimir

AU - Assan, Florence

AU - Chan, Tak Mao

AU - Clarke, Ann Elaine

AU - Crow, Mary K.

AU - Czirják, László

AU - Doria, Andrea

AU - Graninger, Winfried

AU - Halda-Kiss, Bernadett

AU - Hasni, Sarfaraz

AU - Izmirly, Peter M.

AU - Jung, Michelle

AU - Kumánovics, Gábor

AU - Mariette, Xavier

AU - Padjen, Ivan

AU - Pego-Reigosa, José M.

AU - Romero-Diaz, Juanita

AU - Rúa-Figueroa Fernández, Íñigo

AU - Seror, Raphaèle

AU - Stummvoll, Georg H.

AU - Tanaka, Yoshiya

AU - Tektonidou, Maria G.

AU - Vasconcelos, Carlos

AU - Vital, Edward M.

AU - Wallace, Daniel J.

AU - Yavuz, Sule

AU - Meroni, Pier Luigi

AU - Fritzler, Marvin J.

AU - Naden, Ray

AU - Dörner, Thomas

AU - Johnson, Sindhu R.

PY - 2019/9/1

Y1 - 2019/9/1

N2 - Objective: 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). Methods: 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. Results: 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. Conclusion: 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.

AB - Objective: 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). Methods: 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. Results: 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. Conclusion: 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.

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