PT  - JOURNAL ARTICLE
AU  - Martin Aringer
AU  - Ralph Brinks
AU  - Thomas Dörner
AU  - David Daikh
AU  - Marta Mosca
AU  - Rosalind Ramsey-Goldman
AU  - Josef S Smolen
AU  - David Wofsy
AU  - Dimitrios T Boumpas
AU  - Diane L Kamen
AU  - David Jayne
AU  - R Cervera
AU  - Nathalie Costedoat-Chalumeau
AU  - Betty Diamond
AU  - Dafna D Gladman
AU  - Bevra Hahn
AU  - Falk Hiepe
AU  - Søren Jacobsen
AU  - Dinesh Khanna
AU  - Kirsten Lerstrøm
AU  - Elena Massarotti
AU  - Joseph McCune
AU  - Guillermo Ruiz-Irastorza
AU  - Jorge Sanchez-Guerrero
AU  - Matthias Schneider
AU  - Murray Urowitz
AU  - George Bertsias
AU  - Bimba F Hoyer
AU  - Nicolai Leuchten
AU  - Gabriela Schmajuk
AU  - Chiara Tani
AU  - Sara K Tedeschi
AU  - Zahi Touma
AU  - Branimir Anic
AU  - Florence Assan
AU  - Tak Mao Chan
AU  - Ann Elaine Clarke
AU  - Mary K Crow
AU  - László Czirják
AU  - Andrea Doria
AU  - Winfried Graninger
AU  - Bernadett Halda-Kiss
AU  - Sarfaraz Hasni
AU  - Peter M Izmirly
AU  - Michelle Jung
AU  - Gábor Kumánovics
AU  - Xavier Mariette
AU  - Ivan Padjen
AU  - José M Pego-Reigosa
AU  - Juanita Romero-Diaz
AU  - Íñigo Rúa-Figueroa
AU  - Raphaèle Seror
AU  - Georg H Stummvoll
AU  - Yoshiya Tanaka
AU  - Maria G Tektonidou
AU  - Carlos Vasconcelos
AU  - Edward M Vital
AU  - Daniel J Wallace
AU  - Sule Yavuz
AU  - Pier Luigi Meroni
AU  - Marvin J Fritzler
AU  - Ray Naden
AU  - Karen Costenbader
AU  - Sindhu R Johnson
TI  - European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) SLE classification criteria item performance
AID  - 10.1136/annrheumdis-2020-219373
DP  - 2021 Jun 01
TA  - Annals of the Rheumatic Diseases
PG  - 775--781
VI  - 80
IP  - 6
4099  - http://ard.bmj.com/content/80/6/775.short
4100  - http://ard.bmj.com/content/80/6/775.full
SO  - Ann Rheum Dis2021 Jun 01; 80
AB  - Background/objectives The European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) 2019 classification criteria for systemic lupus erythematosus system showed high specificity, while attaining also high sensitivity. We hereby analysed the performance of the individual criteria items and their contribution to the overall performance of the criteria.Methods We combined the EULAR/ACR derivation and validation cohorts for a total of 1197 systemic lupus erythematosus (SLE) and n=1074 non-SLE patients with a variety of conditions mimicking SLE, such as other autoimmune diseases, and calculated the sensitivity and specificity for antinuclear antibodies (ANA) and the 23 specific criteria items. We also tested performance omitting the EULAR/ACR criteria attribution rule, which defines that items are only counted if not more likely explained by a cause other than SLE.Results Positive ANA, the new entry criterion, was 99.5% sensitive, but only 19.4% specific, against a non-SLE population that included other inflammatory rheumatic, infectious, malignant and metabolic diseases. The specific criteria items were highly variable in sensitivity (from 0.42% for delirium and 1.84% for psychosis to 75.6% for antibodies to double-stranded DNA), but their specificity was uniformly high, with low C3 or C4 (83.0%) and leucopenia <4.000/mm³ (83.8%) at the lowest end. Unexplained fever was 95.3% specific in this cohort. Applying the attribution rule improved specificity, particularly for joint involvement.Conclusions Changing the position of the highly sensitive, non-specific ANA to an entry criterion and the attribution rule resulted in a specificity of >80% for all items, explaining the higher overall specificity of the criteria set.All data relevant to this study are included in this manuscript (including the supplementary information) or the previous two manuscripts on the EULAR/ACR classification criteria.