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 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 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

doi:10.1136/annrheumdis-2018-214819

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

^*Corresponding author for this work

Medicine, Rheumatology Division

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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 1001 subjects and validation compared with previous criteria in a new validation cohort of 1270 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 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. 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 reflect current thinking about SLE and provide an improved foundation for SLE research.

Original language	English (US)
Pages (from-to)	1151-1159
Number of pages	9
Journal	Annals of the rheumatic diseases
Volume	78
Issue number	9
DOIs	https://doi.org/10.1136/annrheumdis-2018-214819
State	Published - Sep 1 2019

Funding

61Institute of Health Policy, Management and Evaluation, University of Toronto, Toronto, Ontario, Canada Acknowledgements This body of work was jointly supported by the European League Against Rheumatism and the American College of Rheumatology. One part of the derivation and validation cohort was supported by the Intramural Research Program of the National Institute of Arthritis and Musculoskeletal and Skin Diseases of the National Institutes of Health. The authors wish to acknowledge the diligent work of Banita Aggarwal and Keshini Devakandan in data entry, data cleaning, queries to submitting investigators, data cutting and maintenance of the derivation and validation cohorts; and of Corine Sinnette, MA, in the preparatory work for the multicriteria decision analysis exercise. Funding This study was supported by American College of Rheumatology and European League Against Rheumatism.

Keywords

classification criteria
consensus methods
lupus
multi-criteria decision analysis
systemic lupus erythematosus
validation

ASJC Scopus subject areas

Rheumatology
Immunology and Allergy
Immunology
General Biochemistry, Genetics and Molecular Biology

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Aringer, M., Costenbader, K., Daikh, D., Brinks, R., Mosca, M., Ramsey-Goldman, R., Smolen, J. S., Wofsy, D., Boumpas, D. T., Kamen, D. L., Jayne, D., Cervera, R., Costedoat-Chalumeau, N., Diamond, B., Gladman, D. D., Hahn, B., Hiepe, F., Jacobsen, S., Khanna, D., ... Johnson, S. R. (2019). 2019 European League Against Rheumatism/American College of Rheumatology classification criteria for systemic lupus erythematosus. Annals of the rheumatic diseases, 78(9), 1151-1159. https://doi.org/10.1136/annrheumdis-2018-214819

@article{9bfe58d40f3d437d90c18b1410d579a7,

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 1001 subjects and validation compared with previous criteria in a new validation cohort of 1270 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 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. 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 reflect current thinking about SLE and provide an improved foundation for SLE research.",

keywords = "classification criteria, consensus methods, lupus, multi-criteria decision analysis, systemic lupus erythematosus, validation",

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.}",

note = "Publisher Copyright: {\textcopyright} Author(s) (or their employer(s)) 2019. No commercial re-use. See rights and permissions. Published by BMJ.",

year = "2019",

month = sep,

day = "1",

doi = "10.1136/annrheumdis-2018-214819",

language = "English (US)",

volume = "78",

pages = "1151--1159",

journal = "Annals of the rheumatic diseases",

issn = "0003-4967",

publisher = "BMJ Publishing Group",

number = "9",

}

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', Annals of the rheumatic diseases, vol. 78, no. 9, pp. 1151-1159. https://doi.org/10.1136/annrheumdis-2018-214819

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 1001 subjects and validation compared with previous criteria in a new validation cohort of 1270 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 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. 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 reflect current thinking about SLE and provide 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 1001 subjects and validation compared with previous criteria in a new validation cohort of 1270 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 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. 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 reflect current thinking about SLE and provide an improved foundation for SLE research.

KW - classification criteria

KW - consensus methods

KW - lupus

KW - multi-criteria decision analysis

KW - systemic lupus erythematosus

KW - validation

UR - http://www.scopus.com/inward/record.url?scp=85070711882&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85070711882&partnerID=8YFLogxK

U2 - 10.1136/annrheumdis-2018-214819

DO - 10.1136/annrheumdis-2018-214819

M3 - Article

C2 - 31383717

AN - SCOPUS:85070711882

SN - 0003-4967

VL - 78

SP - 1151

EP - 1159

JO - Annals of the rheumatic diseases

JF - Annals of the rheumatic diseases

IS - 9

ER -

2019 European League Against Rheumatism/American College of Rheumatology classification criteria for systemic lupus erythematosus

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