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

2020 ◽  
Vol 79 (10) ◽  
pp. 1333-1339 ◽  
Author(s):  
Sindhu R Johnson ◽  
Ralph Brinks ◽  
Karen H Costenbader ◽  
David Daikh ◽  
Marta Mosca ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Sensitivity And Specificity ◽  
Lupus Erythematosus ◽  
Disease Duration ◽  
High Sensitivity ◽  
Classification Criteria ◽  
Systemic Lupus ◽  
Early Disease ◽  
Asian Patients ◽  
Acr Criteria

ObjectivesThe 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.MethodsTwenty-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.ResultsThe 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%).ConclusionsThe 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.

THU0271 PERFORMANCE OF THE EULAR/ACR 2019 CLASSIFICATION CRITERIA FOR SYSTEMIC LUPUS ERYTHEMATOSUS IN EARLY DISEASE, ACROSS SEXES AND ETHNICITIES

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 362.1-362
Author(s):  
S. Johnson ◽  
R. Brinks ◽  
K. Costenbader ◽  
D. Daikh ◽  
M. Mosca ◽  
...  
Keyword(s):  
Lupus Erythematosus ◽  
Disease Duration ◽  
Classification Criteria ◽  
Classification Systems ◽  
Early Disease ◽  
Research Support ◽  
Becton Dickinson ◽  
Asian Patients ◽  
Acr Criteria ◽  
New Criteria

Background:EULAR/ACR 2019 SLE Classification Criteria were validated in an international cohort.Objectives:To evaluate performance characteristics of SLE classification systems in sex, race/ethnicity, and disease duration subsets.Methods:Sensitivity and specificity of the EULAR/ACR 2019, SLICC 2012 and ACR 1982/1997 criteria were evaluated in the validation cohort.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; and patients with an SLE duration of 1-3 years (n=196), 3-5 years (n=157), and ≥5 years (n=879). Among patients with 1-3 years disease duration, the EULAR/ACR criteria had better sensitivity than the ACR criteria (97% (95%CI 92-99%) vs 81% (95%CI 72-88%). The new criteria performed well in men (sensitivity 93%, specificity 96%) and women (sensitivity 97%, specificity 94%). The new criteria had better sensitivity than the ACR criteria in White (95% vs 83%), Hispanic (100% vs 86%) and Asian patients (97% vs 77%).Conclusion:The EULAR/ACR 2019 criteria perform well in patients with early disease, and across sexes and ethnicities.Disclosure of Interests:Sindhu Johnson Grant/research support from: Boehringer Ingelheim, Corbus Pharmaceuticals, GlaxoSmithKline, Roche, Merck, Bayer, Consultant of: Boehringer Ingelheim, Ikaria, Ralph Brinks: None declared, Karen Costenbader Grant/research support from: Merck, Consultant of: Astra-Zeneca, David Daikh: None declared, Marta Mosca: None declared, Rosalind Ramsey-Goldman: None declared, Josef S. Smolen Grant/research support from: AbbVie, Eli Lilly, Janssen, Merck Sharp & Dohme, Pfizer, Roche – grant/research support, Consultant of: AbbVie, Amgen Inc., AstraZeneca, Astro, Celgene Corporation, Celtrion, Eli Lilly, Glaxo, ILTOO, Janssen, Medimmune, Merck Sharp & Dohme, Novartis, Pfizer, Roche, Samsung, Sanofi, UCB – consultant, Speakers bureau: AbbVie, Amgen Inc., AstraZeneca, Astro, Celgene Corporation, Celtrion, Eli Lilly, Glaxo, ILTOO, Janssen, Medimmune, Merck Sharp & Dohme, Novartis, Pfizer, Roche, Samsung, Sanofi, UCB – speaker, David Wofsy: None declared, Dimitrios Boumpas Grant/research support from: Unrestricted grant support from various pharmaceutical companies, Diane L Kamen Consultant of: Consulted on SLE survey development for Lilly and consulted on SLE trial protocol development for EMD Serono in 2019, David Jayne Grant/research support from: ChemoCentryx, GSK, Roche/Genentech, Sanofi-Genzyme, Consultant of: Astra-Zeneca, ChemoCentryx, GSK, InflaRx, Takeda, Insmed, Chugai, Boehringer-Ingelheim, Ricard Cervera: None declared, Nathalie Costedoat-Chalumeau Grant/research support from: UCB to my institution, Betty Diamond: None declared, Dafna D Gladman Grant/research support from: AbbVie, Amgen Inc., BMS, Celgene Corporation, Janssen, Novartis, Pfizer, UCB – grant/research support, Consultant of: AbbVie, Amgen Inc., BMS, Celgene Corporation, Janssen, Novartis, Pfizer, UCB – consultant, Bevra H. Hahn Grant/research support from: Janssen Research & Development, LLC, Falk Hiepe: None declared, Soren Jacobsen: None declared, Dinesh Khanna Shareholder of: Eicos Sciences, Inc./Civi Biopharma, Inc., Grant/research support from: Dr Khanna was supported by NIH/NIAMS K24AR063120, Consultant of: Acceleron, Actelion, Bayer, Boehringer Ingelheim, Bristol-Myers Squibb, Corbus Pharmaceuticals, Horizon Therapeutic, Galapagos, Roche/Genentech, GlaxoSmithKline, Mitsubishi Tanabe, Sanofi-Aventis/Genzyme, UCB, Kirsten Lerstrom: None declared, Elena Massarotti: None declared, William Joseph McCune: None declared, Guillermo Ruiz-Irastorza: None declared, Jorge Sanchez-Guerrero: None declared, Matthias Schneider: None declared, Murray B Urowitz: None declared, George Bertsias Grant/research support from: GSK, Consultant of: Novartis, Bimba F. Hoyer: None declared, Nicolai Leuchten: None declared, Chiara Tani: None declared, Sara Tedeschi: None declared, Zahi Touma: None declared, Gabriela Schmajuk Grant/research support from: Pfizer, Branimir Anic: None declared, Florence Assan: None declared, Tak Chan: None declared, Ann E Clarke: None declared, Mary K. Crow: None declared, László Czirják Consultant of: Actelion, BI, Roche-Genentech, Lilly, Medac, Novartis, Pfizer, Bayer AG, Andrea Doria Consultant of: GSK, Pfizer, Abbvie, Novartis, Ely Lilly, Speakers bureau: UCB pharma, GSK, Pfizer, Janssen, Abbvie, Novartis, Ely Lilly, BMS, Winfried Graninger: None declared, Bernadett Halda-Kiss: None declared, Sarfaraz Hasni: None declared, Peter Izmirly: None declared, Michelle Jung: None declared, Gabor Kumanovics Consultant of: Boehringer, Teva, Speakers bureau: Roche, Lilly, Novartis, Xavier Mariette: None declared, Ivan Padjen: None declared, Jose M Pego-Reigosa: None declared, Juanita Romero-Diaz Consultant of: Biogen, Iñigo Rua-Figueroa: None declared, Raphaèle Seror Consultant of: BMS, Medimmune, Novartis, Pfizer, GSK, Lilly, Georg Stummvoll: None declared, Yoshiya Tanaka Grant/research support from: Asahi-kasei, Astellas, Mitsubishi-Tanabe, Chugai, Takeda, Sanofi, Bristol-Myers, UCB, Daiichi-Sankyo, Eisai, Pfizer, and Ono, Consultant of: Abbvie, Astellas, Bristol-Myers Squibb, Eli Lilly, Pfizer, Speakers bureau: Daiichi-Sankyo, Astellas, Chugai, Eli Lilly, Pfizer, AbbVie, YL Biologics, Bristol-Myers, Takeda, Mitsubishi-Tanabe, Novartis, Eisai, Janssen, Sanofi, UCB, and Teijin, Maria Tektonidou Grant/research support from: AbbVie, MSD, Novartis and Pfizer, Consultant of: AbbVie, MSD, Novartis and Pfizer, Carlos Vasconcelos: None declared, Edward Vital Grant/research support from: AstraZeneca, Roche/Genentech, and Sandoz, Consultant of: AstraZeneca, GSK, Roche/Genentech, and Sandoz, Speakers bureau: Becton Dickinson and GSK, Daniel J Wallace: None declared, Sule Yavuz: None declared, Pier Luigi Meroni: None declared, Marvin Fritzler: None declared, Raymond Naden: None declared, Thomas Dörner Grant/research support from: Janssen, Novartis, Roche, UCB, Consultant of: Abbvie, Celgene, Eli Lilly, Roche, Janssen, EMD, Speakers bureau: Eli Lilly, Roche, Samsung, Janssen, Martin Aringer Consultant of: Boehringer Ingelheim, Roche, Speakers bureau: Boehringer Ingelheim, Roche

scholarly journals FRI0551 PERFORMANCE OF THE 2019 AMERICAN COLLEGE OF RHEUMATOLOGY/EUROPEAN LEAGUE AGAINST RHEUMATISM SYSTEMIC LUPUS ERYTHEMATOSUS CLASSIFICATION CRITERIA

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 877.2-877
Author(s):  
C. Vrancianu ◽  
I. Conea ◽  
A. Boca ◽  
M. Bolboceanu ◽  
C. Draganesscu ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Sensitivity And Specificity ◽  
Lupus Erythematosus ◽  
Disease Duration ◽  
Classification Criteria ◽  
Control Group ◽  
Systemic Lupus ◽  
American College Of Rheumatology ◽  
European League Against Rheumatism ◽  
European League

Background:Systemic lupus erythematosus (SLE) is a heterogenous autoimmune disease, with increased morbidity and mortality, often diagnosed in advanced stages. The recently published 2019 American College Of Rheumatology/European League Against Rheumatism (ACR/EULAR) classification criteria for SLE are weighted, hierarchically clustered criteria developed to increase reliability and the identification of early SLE.Objectives:To compare the sensitivity and specificity of the 2019 ACR/EULAR criteria with the 2012 SLICC criteria in a large single-centre cohort of patients with SLE, diagnosed according to expert oppinion.Methods:Data of SLE patients evaluated in our centre between 1996-2019 have been retrospectively analyzed. The control cohort included patients with positive antinuclear antibodies of other ethiology than SLE, evaluated between 2001-2019. The sensitivity and specificity of the 2019 ACR/EULAR and 2012 SLICC criteria were tested using the McNemar test for correlated proportions.Results:Four hundred and forty-six patients with SLE (413 women, mean±SD age 40.5±12.7 years, disease duration 10.1±9.2 years) and 67 controls (63 women, mean±SD age 50.4±12.6 years, disease duration 7.6±6.9 years; 29 systemic sclerosis (SSc), 18 mixed connective tissue dissease (MCTD), 15 undifferentiated CTD, 2 rheumatoid arthritis (RA), 2 SSc – RA overlaps and 1 dermatomyositis) were included. The sensitivity of the 2019 ACR/EULAR and 2012 SLICC criteria were similar 85.4% and 83.6 %, respectively (p=0.3). The specificity of the 2019 ACR/EULAR and 2012 SLICC criteria were 70.2 % and 86.6%, respectively (p=0.007). In the SLE group, patients missclassified according to the new 2019 ACR/EULAR criteria were 65, whereas according to the 2012 SLICC criteria were 73; of them, 44 patients did not fulfill any criteria. In the control group, patients misclassified had mainly MCTD (13/20 patients according to the new 2019 ACR/EULAR, and 8/9 according to the 2012 SLICC criteria).Conclusion:In this real-life cohort, the 2019 ACR/EULAR criteria have a similar sensitivity and lower specificity than the 2012 SLICC criteria, misclassifying especially MCTD patients. These results might be due to the long disease duration in our cohort.References:[1] Aringer M, Costenbader K, Daikh D, et al. 2019 European League Against Rheumatism/American College of Rheumatology Classification Criteria for Systemic Lupus Erythematosus. Arthritis Rheumatol. 2019;71(9):1400–1412. doi:10.1002/art.40930Disclosure of Interests:None declared

scholarly journals Assessment of EULAR/ACR-2019, SLICC-2012 and ACR-1997 Classification Criteria in SLE with Longstanding Disease

2021 ◽  
Vol 10 (11) ◽  
pp. 2377
Author(s):  
Berta Magallares ◽  
David Lobo-Prat ◽  
Ivan Castellví ◽  
Patricia Moya ◽  
Ignasi Gich ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Nephritis ◽  
Lupus Erythematosus ◽  
Antiphospholipid Antibodies ◽  
Disease Duration ◽  
Renal Involvement ◽  
Damage Index ◽  
High Sensitivity ◽  
Classification Criteria ◽  
Systemic Lupus

Background: Different classification criteria for systemic lupus erythematosus (SLE) have been launched over the years. Our aim was to evaluate the performance of the EULAR/ACR-2019, SLICC-2012 and ACR-1997 classification criteria in a cohort of SLE patients with longstanding disease. Methods: Descriptive observational study in 79 patients with established and longstanding SLE. The three classification criteria sets were applied to those patients. Results: Of the 79 patients, 70 were women (88.6%), with a mean age of 51.8 ± 14 years and a mean disease duration of 15.2 ± 11.5 years. The sensitivity of the different criteria were: 51.9%, 87.3% and 86.1% for ACR-1997, SLICC-2012 and EULAR/ACR-2019, respectively. In total, 68 out of 79 patients (53.7%) met all three classification criteria; 11.4% did not meet any classification criteria and were characterized by low SLEDAI (0.6 ± 0.9), low SLICC/ACR Damage Index (0.88 ± 0.56) and fulfilling only skin domains, antiphospholipid antibodies or hypocomplementemia. To fulfill EULAR/ACR-2019 criteria was associated with low complement levels (p < 0.04), high anti-dsDNA levels (p < 0.001), presence of lupus nephritis III-IV (p < 0.05) and arthritis (p < 0.001). Conclusion: The EULAR/ACR-2019 classification criteria showed high sensitivity, similar to SLICC-2012, in SLE patients with longstanding disease. Patients with serological, articular or renal involvement are more likely to fulfill SLICC-2012 or EULAR/ACR-2019 criteria.

scholarly journals POS0706 PERFORMANCES OF DIFFERENT CLASSIFICATION CRITERIA FOR SYSTEMIC LUPUS ERYTHEMATOSUS IN A SINGLE CENTER COHORT FROM TURKEY

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 602.1-603
Author(s):  
E. S. Torun ◽  
E. Bektaş ◽  
F. Kemik ◽  
M. Bektaş ◽  
C. Cetin ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Positive Predictive Value ◽  
Negative Predictive Value ◽  
Antiphospholipid Syndrome ◽  
Lupus Erythematosus ◽  
Predictive Value ◽  
Sjogren's Syndrome ◽  
Classification Criteria ◽  
Systemic Lupus ◽  
Acr Criteria

Background:Recently developed EULAR/ACR classification criteria for systemic lupus erythematosus (SLE) have important differences compared to the 2012 Systemic Lupus International Collaborating Clinics (SLICC) SLE classification criteria and the revised 1997 American College of Rheumatology (ACR) criteria: The obligatory entry criterion of antinuclear antibody (ANA) positivity is introduced and a “weighted” approach is used1. Sensitivity and specificity of these three criteria have been debated and may vary in different populations and clinical settings.Objectives:We aim to compare the performances of three criteria sets/rules in a large cohort of patients and relevant diseased controls from a reference center with dedicated clinics for SLE and other autoimmune/inflammatory connective tissue diseases from Turkey.Methods:We reviewed the medical records of SLE patients and diseased controls for clinical and laboratory features relevant to all sets of criteria. Criteria sets/rules were analysed based on sensitivity, positive predictive value, specificity and negative predictive value, using clinical diagnosis with at least 6 months of follow-up as the gold standard. A subgroup analysis was performed in ANA positive patients for both SLE patients and diseased controls. SLE patients that did not fulfil 2012 SLICC criteria and 2019 EULAR/ACR criteria and diseased controls that fulfilled these criteria were evaluated.Results:A total of 392 SLE patients and 294 non-SLE diseased controls (48 undifferentiated connective tissue disease, 51 Sjögren’s syndrome, 43 idiopathic inflammatory myopathy, 50 systemic sclerosis, 52 primary antiphospholipid syndrome, 15 rheumatoid arthritis, 15 psoriatic arthritis and 20 ANCA associated vasculitis) were included into the study. Hundred and fourteen patients (16.6%) were ANA negative.Sensitivity was more than 90% for 2012 SLICC criteria and 2019 EULAR/ACR criteria and positive predictive value was more than 90% for all three criteria (Table 1). Specificity was the highest for 1997 ACR criteria. Negative predictive value was 76.9% for ACR criteria, 88.4% for SLICC criteria and 91.7% for EULAR/ACR criteria.In only ANA positive patients, sensitivity was 79.6% for 1997 ACR criteria, 92.2% for 2012 SLICC criteria and 96.1% for 2019 EULAR/ACR criteria. Specificity was 92.6% for ACR criteria, 87.8% for SLICC criteria 85.2% for EULAR/ACR criteria.Eleven clinically diagnosed SLE patients had insufficient number of items for both 2012 SLICC and 2019 EULAR/ACR criteria. Both criteria were fulfilled by 16 diseased controls: 9 with Sjögren’s syndrome, 5 with antiphospholipid syndrome, one with dermatomyositis and one with systemic sclerosis.Table 1.Sensitivity, positive predictive value, specificity and negative predictive value of 1997 ACR, 2012 SLICC and 2019 EULAR/ACR classification criteriaSLE (+)SLE (-)Sensitivity (%)Positive Predictive Value (%)Specificity (%)Negative Predictive Value (%)1997 ACR(+) 308(-) 841527978.695.494.976.92012 SLICC(+) 357(-) 352626891.193.291.288.42019 EULAR/ACR(+) 368(-) 242826693.892.990.591.7Conclusion:In this cohort, although all three criteria have sufficient specificity, sensitivity and negative predictive value of 1997 ACR criteria are the lowest. Overall, 2019 EULAR/ACR and 2012 SLICC criteria have a comparable performance, but if only ANA positive cases and controls are analysed, the specificity of both criteria decrease to less than 90%. Some SLE patients with a clinical diagnosis lacked sufficient number of criteria. Mostly, patients with Sjögren’s syndrome or antiphospholipid syndrome are prone to misclassification by both recent criteria.References:[1]Aringer M, Costenbader K, Daikh D, et al. 2019 European League Against Rheumatism/American College of Rheumatology classification criteria for systemic lupus erythematosus. Ann Rheum Dis 2019;78:1151-1159.Disclosure of Interests:None declared

SAT0217 PERFORMANCE OF ACR/EULAR 2019, SLICC 2012 AND ACR 1997 CLASSIFICATION CRITERIA IN A COHORT OF SYSTEMIC LUPUS ERYTHEMATOSUS PATIENTS WITH LONGSTANDING DISEASE

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1051-1052
Author(s):  
D. Lobo Prat ◽  
B. Magallares ◽  
I. Castellví ◽  
H. Park ◽  
P. Moya ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Statistical Significance ◽  
Renal Involvement ◽  
High Sensitivity ◽  
Classification Criteria ◽  
Average Score ◽  
Systemic Lupus ◽  
Malar Rash ◽  
Oral Ulcers

Background:Systemic lupus erythematosus (SLE) is an autoimmune disease with variable clinical features and a complex physiopathology. In 2019, EULAR and ACR have jointly developed new classification criteria with both high sensitivity and specificity. These criteria have the particularity of including the presence of ANA as an obligatory entry criterion and the existence of clinical and immunological domains with weighted scores.Objectives:To evaluate the performance and characteristics of the ACR/EULAR 2019, SLICC 2012 and ACR 1997 classification criteria in a cohort of SLE patients with longstanding disease.Methods:Descriptive observational study that enrolled a cohort of SLE patients with longstanding disease followed in a tertiary level hospital. Demographic and clinical data were gathered along with the fulfillment of classification criteria. The sensitivity of each classification criteria and the statistically significant associations between criteria fulfillment and clinical and immunological data were calculated. Statistical analyses were performed using the Chi2, T-student and ANOVA tests. Statistical significance was assumed in p values <0.05.Results:A total of 79 patients (88.6% women) with a mean age of 51.8±14 years, disease duration of 15.2±11.5 years and SLEDAI of 2.65±2.1 were included. The sensitivity of the different classification criteria was 51.9% for ACR 1997, 87.3% for SLICC 2012 and 86.1% for ACR/EULAR 2019 (Table 1).Table 1.Sensitivity and average scores.ACR/EULAR 2019SLICC 2012ACR 1997Sensitivity (%)86.187.351.9Average score of patients classified as SLE(±SD)18.6±5.85.3±1.45±0.9Average score of patients NOT classified as SLE(±SD)6.1±2.52.8±0.42.8±0.851.9% of patients met all three classification criteria, 29.1% met SLICC 2012 and ACR/EULAR 2019, 5% only met SLICC 2012 and 3.7% exclusively met ACR/EULAR 2019. 11.4% of patients did not meet any classification criteria and were characterized by having a low SLEDAI (0.6±0.9) and fulfilling only skin domains (alopecia or oral ulcers), antiphospholipid antibodies or hypocomplementemia.Statistically significant associations were found between meeting ACR/EULAR 2019 classification criteria and the presence of low C3 and C4 (p<0.04), DNA (p<0.001), lupus nephritis III-IV (p<0.05) and arthritis (p<0.001), highlighting that all patients with arthritis met these criteria.In the SLICC 2012 evaluation, significant associations were found between meeting these criteria and the presence of arthritis (p<0.01), renal involvement (p<0.04), leukopenia/lymphopenia (p=0.05), DNA (p<0.03) and hypocomplementemia (p=0.02).Fullfilment of ACR 1997 was associated to the presence of malar rash (p<0.001), discoid lupus (p<0.05), photosensitivity (p<0.001) and oral ulcers (p<0.04), as well as arthritis (p<0.001), serositis (p=0.02), renal (p<0.05) and hematologic (p=0.05) involvement.The Kappa concordance coefficient among classification criteria is detailed in Table 2.Table 2.Kappa concordance coefficient.ACR/EULAR 2019 - SLICC 2012ACR/EULAR 2019 - ACR 1997SLICC 2012 - ACR 1997Kappa concordance coefficient0.610.270.30Conclusion:The ACR/EULAR 2019 classification criteria maintain a high sensitivity similar to the SLICC 2012 in SLE patients with longstanding disease, both of which are much higher than ACR 1997. Patients with serological, articular or renal involvement are more likely to meet SLICC 2012 or ACR/EULAR 2019 criteria. It is noteworthy the relevance of dermatological manifestations in ACR1997 classification criteria against the increased weight that a better understanding of SLE physiopathology has provided to analytic and immunological criteria in the subsequent classification criteria.Disclosure of Interests:David Lobo Prat: None declared, Berta Magallares: None declared, Ivan Castellví Consultant of: Boehringer Ingelheim, Actelion, Kern Pharma, Speakers bureau: Boehringer Ingelheim, Actelion, Bristol-Myers Squibb, Roche, HyeSang Park: None declared, Patricia Moya: None declared, Ignasi Gich: None declared, Ana Laiz: None declared, Cesar Díaz-Torné: None declared, Ana Milena Millán Arciniegas: None declared, Susana P. Fernandez-Sanchez: None declared, Hector Corominas: None declared

A comparative study on clinical and serological characteristics between patients with rhupus and those with systemic lupus erythematosus and rheumatoid arthritis

Lupus ◽  
2020 ◽  
Vol 29 (10) ◽  
pp. 1216-1226
Author(s):  
Beatriz Frade-Sosa ◽  
Javier Narváez ◽  
Tarek Carlos Salman-Monte ◽  
Raul Castellanos-Moreira ◽  
Vera Ortiz-Santamaria ◽  
...  
Keyword(s):  
Rheumatoid Arthritis ◽  
Systemic Lupus Erythematosus ◽  
Autoimmune Diseases ◽  
Lupus Erythematosus ◽  
Disease Duration ◽  
Renal Involvement ◽  
Clinical Manifestations ◽  
Classification Criteria ◽  
Systemic Lupus ◽  
Cross Sectional

Background The concomitant presence of two autoimmune diseases – systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA) – in the same patient is known as rhupus. We evaluated a group of patients with rhupus to clarify further their clinical, serological and immunogenic features in a multi-centre cohort. In addition, the study aimed to explore the utility of the 2019 European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) SLE classification criteria in our group of patients with rhupus. Methods This was a cross-sectional study. We included rhupus patients from 11 different rheumatology departments, and compared them to SLE and RA patients at a ratio of 2:1. All information was recorded following a pre-established protocol. Results A total of 200 patients were included: 40 rhupus patients and 80 each of SLE and RA patients as controls. Disease duration was similar among SLE and rhupus groups (around 13 years), but the RA group had a significantly lower disease duration. Main clinical manifestations were articular (94.2%), cutaneous (77.5%) and haematological (72.5%). Rhupus patients had articular manifestations similar to those expected in RA. Only 10% of rhupus patients had renal involvement compared with 25% of those with SLE ( p < 0.05), while interstitial lung disease was more common in patients affected by RA. The 2019 EULAR/ACR SLE criteria were met in 92.5% of the rhupus patients and in 96.3% of the SLE cohort ( p > 0.05). Excluding the joint domain, there were no differences between the numbers of patients who met the classification criteria. Conclusion Rhupus patients follow a particular clinical course, with full expression of both SLE and RA in terms of organ involvement, except for a lower prevalence of kidney affection. The new 2019 EULAR/ACR SLE criteria are not useful for differentiating SLE and rhupus patients. A new way of classifying autoimmune diseases is needed to identify overlapping clusters.

scholarly journals New Classification Criteria for Systemic Lupus Erythematosus

2020 ◽  
Vol 95 (3) ◽  
pp. 151-161
Author(s):  
Yeon-Ah Lee
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Clinical Findings ◽  
Classification Criteria ◽  
Systemic Lupus ◽  
American College Of Rheumatology ◽  
Acr Criteria ◽  
European League Against Rheumatism ◽  
Sum Score

Systemic lupus erythematosus (SLE) is a prototypic autoimmune disease with highly variable clinical and immunological manifestations. Classification and diagnosis of SLE are complicated by the multi-organ nature of the disease and by our incomplete understanding of its pathophysiology. The 1997 update of the 1982 American College of Rheumatology (ACR) criteria for SLE has been widely used for classification of SLE. In order to improve clinical relevance and early diagnosis, the Systemic Lupus Erythematosus International Collaborating Clinics (SLICC) group suggested the 2012 SLICC criteria. These sets of classification criteria have unweighted lists of various serological and clinical findings typical of SLE, can be fulfilled by reaching a sum score of points. The only exception is biopsy-proven lupus nephritis with autoantibodies in the 2012 SLICC criteria. In an attempt to overcome limitations of the previous sets of SLE classification criteria, the new 2019 SLE European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) classification criteria for SLE have been recently published. The 2019 EULAR/ACR criteria include positive ANA at least once as obligatory entry criterion; followed by additive hierarchically clustered and weighted criteria. The structure and weighting of criteria constitute a paradigm shift in the classification of SLE. In the validation cohort, the new criteria had a sensitivity of 96.1% and specificity of 93.4%. This review attempts to delineate the history, performance and limitations of the current sets of SLE criteria.

Application of SLICC classification criteria in undifferentiated connective tissue disease and evolution in systemic lupus erythematosus: analysis of a large monocentric cohort with a long-term follow-up

Lupus ◽  
2016 ◽  
Vol 26 (6) ◽  
pp. 616-622 ◽  
Author(s):  
A Bortoluzzi ◽  
F Furini ◽  
F Campanaro ◽  
M Govoni
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Connective Tissue ◽  
Lupus Erythematosus ◽  
Classification Criteria ◽  
Undifferentiated Connective Tissue Disease ◽  
Systemic Lupus ◽  
Acr Criteria ◽  
Long Term Follow Up

Objectives The objectives of this study were to analyse the performance of the Systemic Lupus International Collaborating Clinics (SLICC) 2012 classification criteria for systemic lupus erythematosus (SLE) in a large cohort of undifferentiated connective tissue disease (UCTD) population at onset of the disease and during a long-term follow-up of 15 years (1999–2013) and to evaluate the transition from UCTD to SLE, according to American College of Rheumatology (ACR) 1997 and SLICC 2012 classification criteria. Methods A cohort of patients who met the classification criteria proposed by Mosca et al. for UCTD, were analysed. The SLICC 2012 classification criteria for SLE were retrospectively applied to each patient at the time of the diagnosis (T0) and also periodically re-applied and compared to ACR 1997 criteria at three different time points in the follow-up. Results 329 patients were enrolled. According to inclusion criteria at T0 no patient met the SLE/ACR criteria, whilst, retrospectively applying the SLE/SLICC criteria, 44 patients already satisfied this set of criteria for SLE. During the follow-up 23 new patients reached the SLE/SLICC criteria and 14 patients met the ACR criteria with a stable rate of progression to SLE over time. Acute or subacute skin rash, antiphospholipid antibody (aPL) positivity and serositis were the variables correlated to the evolution to SLE. Conclusions In our UCTD population, the application of SLICC classification criteria for SLE at disease onset allowed identification of a proportion of otherwise missed SLE cases; during follow-up, and compared with ACR criteria, SLICC criteria expanded the number of patients classifiable as SLE otherwise classified as UCTD.

scholarly journals POS0763 PERFORMANCE OF THE NEW ACR/EULAR 2019 CLASSIFICATION CRITERIA FOR SYSTEMIC LUPUS ERYTHEMATOSUS (SLE) IN A COHORT OF ARGENTINIAN PATIENTS

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 635.1-635
Author(s):  
J. M. Dapeña ◽  
E. R. Serrano ◽  
J. M. Bande ◽  
M. A. Medina ◽  
D. S. Klajn ◽  
...  
Keyword(s):  
Sensitivity And Specificity ◽  
Lupus Erythematosus ◽  
Gold Standard ◽  
Antiphospholipid Antibodies ◽  
Disease Duration ◽  
Connective Tissue Diseases ◽  
Classification Criteria ◽  
Control Group ◽  
Case Group ◽  
Systemic Lupus

Background:In 2019 ACR and EULAR published in joint collaboration the new classification criteria for Systemic Lupus Erythematosus (SLE). Compared to the previous ones, these criteria have shown higher sensitivity and specificity in multiple cohorts. To our knowledge, its performance has not been evaluated in a cohort of patients with rheumatological diseases living in Argentina.Objectives:The aim of this study was to evaluate the sensitivity and specificity of the ACR/ EULAR 2019 criteria in a cohort of patients with connective tissue diseases residing in Argentina. Secondary objectives were to determine the Likelihood Ratio (LR) of these criteria and the correlation of their global score with activity and damage indexes of the disease.Methods:Multicentre, retrospective and analytical study. Patients ≥ 18 years old with diagnosis of SLE (ACR 1997/SLICC 2012) without other associated collagen diseases (case group), and patients with other non-SLE connective tissue diseases (control group) were included. Those with active infectious disease, oncohematological disease, drug-induced lupus and overlap syndrome were excluded. Sociodemographic data, characteristics of the disease and treatment were recorded. In addition, activity and damage indexes were recorded in the group with SLE.Three SLE experts, blinded to the diagnosis determined, for every individual if the patient had SLE or another rheumatological disease. An interrater agreement of 100% (including the 3 evaluators) was considered “defined SLE” and used as gold standard. In all cases, ACR 1997/SLICC 2012/ACR / EULAR 2019 criteria were applied and compared with the gold standard. Statistical analysis: Descriptive statistics was estimated. Sensitivity, specificity, positive and negative LR of the criteria were determined. The association between the final score of the ACR-EULAR 2019 criteria and the disease activity and damage indexes were estimated with Spearman correlation test. STATA 15.0 was used for data analysis.Results:A total of 365 patients from 7 centres in Argentina were included. A One hundred and eighty-three belonged to the SLE group: 92.3% women, mean age 39 years (SD 13.3), median disease duration 92 months (IQR 37-150). The most frequent manifestations of the disease were mucocutaneous (94%), musculoskeletal (82.5%) and haematological (69%). All patients presented ANA +, 88% hypocomplementemia, 69.4% Anti-DNA and 19.5% antiphospholipid antibodies. Median SLEDAI and SLICC were 2 (IQR 0-6) and 0 (IQR 0-1), respectively.In the control group, 182 patients were recruited: 84% women, mean age 53.6 years (SD 14.2) and median disease duration 82.5 months (IQR 38-151). The most frequent diseases were Rheumatoid Arthritis (46.1%), Scleroderma (18.1%) and Sjögren’s Syndrome (16.5%) and most common manifestations were musculoskeletal (81.9%), immunological (73.6%) and constitutional (25.3%). A total of 62.6% of patients presented ANA+, 8.6% hypocomplementemia, and 1.3% Antiphospholipid antibodies.Ninety-one percent of patients in the case group were classified as defined SLE and 3.8% in the control group.The ACR / EULAR 2019 Criteria showed a 99.4% sensitivity and an 89.1% specificity, with a LR+ of 9.1 and a LR- of 0.007. The sensitivity and specificity of SLICC 2012 criteria were 98.3% and 88%, respectively with a LR+ of 8.2 and a LR- of 0.02; and the ACR 1997 criteria showed a 93.96% sensitivity and 90.1% specificity, with LR + of 8.21 and LR - of 0.07.The correlations between the ACR/EULAR 2019 Criteria global score, and activity and damage indexes were 0.19 and -0.006, respectively.Conclusion:The new ACR / EULAR 2019 criteria have shown high sensitivity, a specificity comparable to its predecessors, and a higher ability to distinguish SLE from other diseases and to exclude it in non-SLE patients. No correlation was observed between the criteria scores and activity and damage indexes.References:[1]Aringer M, Costenbader K, Daikh D, et al 2019 EULAR/ACR classification criteria for SLE. Ann Rheum 2019; 78: 1151-1159.Disclosure of Interests:None declared

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