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 ◽  
...  

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

2020 ◽  
Vol 79 (10) ◽  
pp. 1333-1339 ◽  
Author(s):  
Sindhu R Johnson ◽  
Ralph Brinks ◽  
Karen H Costenbader ◽  
David Daikh ◽  
Marta Mosca ◽  
...  

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.


Rheumatology ◽  
2021 ◽  
Vol 60 (Supplement_1) ◽  
Author(s):  
Reem Abdwani ◽  
Eiman Al Masroori ◽  
Eiman Abdalla ◽  
Safiya Al Abrawi ◽  
Ibrahim Al Zakwani

Abstract Background/Aims  The aim of the study is to compare the performance characteristics among three SLE classification criteria (ACR-1997, SLICC-2012 and EULAR/ACR-2019) in childhood onset SLE (cSLE) cohort of Arab ethnicity from Oman. Methods  We conducted a retrospective multicenter study among pediatric rheumatology centers in Oman. Cases were cSLE and controls were patients with other rheumatic disease with a positive ANA titer followed up over the past 10 years. Data were retrospectively collected to establish the ACR, SLICC and EULAR/ACR criteria fulfilled at first visit, first year follow up and last year of follow up. Results  Study population included 113 cSLE cases (mean age at diagnosis 7.3 ± 3.4 years with disease duration 6.13± 4.6 years and 38% family history of SLE) and 51 controls (mean age at diagnosis 5.0 ± 3.4 years with disease duration 5.7 ± 3.9 years and 4% family history of SLE). Table 1 demonstrates the result of the performance measures for the ACR, SLICC and EULAR/ACR criteria at first visit, first year and last follow up. P123 Table 1:Performance measures for the ACR 1997, SLICC 2012 and ACR/EULAR 2019 classification criteria according to first visit, first year and latest periods.CriteriaSensitivity (95% CI)Specificity (95% CI)PPV (95% CI)NPV (95% CI)ROC (95% CI)Accuracy (95% CI)ACR 19971st visit49% (40%-59%)96% (87%-99%)97% (88%-99%)46% (36%-56%)0.73 (0.67-0.78)64% (56%-71%)1st year57% (47-66%)96% (87%-99%)97% (90%-99%)50% (40%-60%)0.76 (0.71-0.82)69% (61%-76%)Latest66% (56%-74%)96% (87%-99%)97% (91%-99%)56% (45%-66%)0.81 (0.76-0.86)75% (68%-81%)SLICC1st visit76% (67%-84%)94% (84%-99%)97% (91%-99%)64% (52%-75%)0.85 (0.80-0.90)82% (75%-87%)1st year84% (76%-90%)94% (84%-99%)97% (91%-99%)73% (60%-83%)0.89 (0.84-0.94)87% (81%-92%)Latest86% (78%-92%)94% (84%-99%)97% (92%-99%)75% (63%-85%)0.90 (0.85-0.95)88% (83%-93%)ACR/EULAR1st visit81% (73%-88%)92% (81%-98%)96% (90%-99%)69% (57%-80%)0.87 (0.82-0.92)85% (78%-90%)1st year88% (80%-93%)90% (79%-97%)95% (89%-98%)77% (64%-87%)0.89 (0.84-0.94)88% (83%-93%)Latest89% (82%-94%)90% (79%-97%)95% (89%-99%)79% (67%-89%)0.90 (0.85-0.95)90% (84%-94%)ACR/EULAR*1st visit76% (67%-84%)96% (87%-99%)98% (92%-99%)65% (53%-75%)0.86 (0.81-0.91)83% (76%-88%)1st year83% (74%-89%)96% (87%-99%)98% (93%-99%)71% (59%-81%)0.89 (0.85-0.94)87% (81%-92%)Latest84% (76%-90%)96% (87%-99%)98% (93%-99%)73% (61%-83%)0.90 (0.86-0.94)88% (83%-93%)ACR, American College of Rheumatology 1997; SLICC, Systemic Lupus Erythematosus International Collaborating Clinics; EULAR, European League Against Rheumatism; CI, confidence interval; PPV, positive predictive value; NPV, negative predictive value; ROC, area under the received operating curve. ACR/EULAR*, a score of ≥ 13 as compared to the traditional cut of score of ≥ 10. The median disease duration was 5 (2.5-9) years with a range of 1-19 years. Conclusion  In this cSLE population, the EULAR/ACR criteria scored better in sensitivity at first, one year and last follow up; while the ACR 1997 scored better in specificity at first, one year and last follow up. However, if the EULAR/ACR cut score ≥ 13 (rather than traditional cut-score ≥ 10) then it an equivalent specificity to ACR 1997 at the expense of lower sensitivity. Disclosure  R. Abdwani: None. E. Al Masroori: None. E. Abdalla: None. S. Al Abrawi: None. I. Al Zakwani: None.


2011 ◽  
Vol 70 (6) ◽  
pp. 949-955 ◽  
Author(s):  
Mohammed Z Cader ◽  
Andrew Filer ◽  
Jonathan Hazlehurst ◽  
Paola de Pablo ◽  
Christopher D Buckley ◽  
...  

ObjectiveEarly identification of patients with rheumatoid arthritis (RA) is essential to allow the prompt institution of therapy. The 2010 American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) classification criteria, which replace the 1987 classification criteria, have been developed to facilitate such identification in patients with newly presenting inflammatory arthritis. This study therefore assesses the performance of these new criteria in patients with early synovitis.MethodsData were analysed from patients with synovitis seen within 3 months of the onset of inflammatory arthritis. Patients were followed for 18 months to determine outcomes, and data on the cumulative fulfilment of 2010 and 1987 criteria and therapy were recorded.Results265 patients were included in the study. 60 had alternative diagnoses at baseline. Of the remaining 205 patients, 20% fulfilled both 1987 and 2010 criteria, 3% fulfilled only 1987 criteria and 22% fulfilled only 2010 criteria at baseline. The 2010 criteria, when applied at baseline, detected more patients who eventually required disease-modifying antirheumatic drugs (DMARD) (65 (62%) vs 40 (38%); p<0.001), especially methotrexate (50 (68%) vs 31 (42%); p<0.01), within the first 18 months. However, more patients whose disease eventually resolved without ever requiring DMARD were classified at baseline as RA according to the 2010 criteria than with the 1987 criteria (16 (8%) vs 5 (2%); p=0.01).ConclusionThe 2010 ACR/EULAR criteria allow more rapid identification of patients requiring methotrexate compared with the 1987 ACR criteria when applied at baseline. However, overdiagnosis is an important issue to consider if these criteria are to be used in very early disease.


2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 602.1-603
Author(s):  
E. S. Torun ◽  
E. Bektaş ◽  
F. Kemik ◽  
M. Bektaş ◽  
C. Cetin ◽  
...  

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


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 ◽  
...  

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.


Cephalalgia ◽  
2008 ◽  
Vol 28 (3) ◽  
pp. 296-299 ◽  
Author(s):  
R Davey ◽  
J Bamford ◽  
P Emery

Headache is a common symptom described by patients with systemic lupus erythematosus (SLE). It is uncertain whether both the prevalence and phenotype of headache disorders seen in patients with SLE are similar to those in the general population. The current American College of Rheumatology (ACR) classification of headache disorders includes only five categories, included ‘Intractable headache, non-specific’, which is not further defined. The International Headache Society (IHS) has produced a classification which aims to include all recognized headache disorders. We compared the performance of the IHS and ACR criteria in 61 subjects with SLE. Whereas reference to the IHS criteria enabled classification of all headache disorders seen in the cohort, use of the ACR criteria resulted in failure to classify 22% of headache disorders. We suggest that the ACR criteria require revision. Until this is done, IHS criteria should be used in all future studies of headache in SLE.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 358.3-358
Author(s):  
L. Delaval ◽  
T. Goulenok ◽  
A. Dossier ◽  
T. Papo ◽  
K. Sacre

Background:The new 2019 SLE European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) classification criteria for systemic lupus erythematosus (SLE) have been recently published. Seritis is a prominent -often inaugural- feature of active SLE. Low titers of antinuclear antibodies (ANA) have been frequently reported in patients with idiopathic pericarditis. Of note, ANA positivity at a titer ≥1/80 is now mandatory as an entry criterion in the 2019 SLE EULAR/ACR classification criteria.Objectives:Although classification criteria have theoretically no individual diagnostic purpose, we aimed at testing this new criteria set in unselected patients with pericardial effusionMethods:In a retrospective study performed in the Department of Internal Medicine, University Paris Diderot, a French competence centre for rare systemic autoimmune diseases (AID), all consecutive adult patients hospitalized from January 2009 to January 2019 for pericardial effusion were reviewed. Clinical and biological data collected at time of the diagnosis of pericardial effusion were analyzed. The characteristics of the patients are listed in Table 1. Three sets of lupus criteria (SLE ACR-1997, SLE SLICC and 2019 SLE EULAR/ACR criteria) were applied in all ANA-positive patientsResults:Over a 10-year period, 137 patients were admitted for pericardial effusion. Search for ANA was systematically performed at diagnosis in all but 8 (n=129) and measured at a titer ≥ 1:80 on Hep-2 cells in 49 patients (38%) that were eventually separated in three groups: 17 (34.7%) patients with a final diagnosis of SLE based on senior clinician judgement, 6 (12.2%) patients with a final diagnosis of autoimmune disease (AID) other than SLE (primary Sjögren’s syndrome (n=2), undifferentiated connective-tissue disease (n=2) and systemic sclerosis (n=2)) and 26 (53.1%) patients with a diagnosis of idiopathic pericarditis after exclusion of malignancy, tuberculosis and systemic inflammatory diseases with a median 12.3 [1.6-29.8] months follow-upThe 2019 SLE EULAR/ ACR criteria were met in 100% of patients with SLE, 33.3% of patients with non-SLE AID and 11.5% of patients with idiopathic pericarditis. Thus this new set of criteria for SLE offered a higher sensitivity (100%) but a lower specificity (84.38%) as compared to the former criteria, for the diagnosis of SLE in patients with pericardial effusion. Interestingly, the 2019 SLE EULAR/ACR classification score was higher in SLE patients (median: 30 [11-45]) as compared to non-SLE AID (median: 8 [6-12], p=0.0006) and idiopathic pericarditis patients (median: 6 [5-12], p< 0.00001). Moreover, the 2019 classification set score strongly correlated with the SLEDAI activity score [6] as shown Figure S1 (R2=0.8105, p<0.00001). Setting the 2019 SLE EULAR/ACR classification threshold score >12 (out of a theoretical maximum of 51) instead of ≥10 increased the specificity of 2019 SLE EULAR/ ACR criteria from 84.38% to 100%. Overall, in patients with pericardial effusion and positive ANA, the diagnosis of SLE could be ruled out when 2019 SLE EULAR/ACR criteria score was < 10 and confirmed when the score was > 12.Conclusion:This study shows that the new 2019 SLE EULAR/ACR criteria for SLE are helpful in clinical practice for the diagnosis of SLE in patients admitted for pericardial effusionAcknowledgments:Jean-François Alexandra, Marie Berleur, Marie-Paule Chauveheid, Gregory Ducrocq, Damien van Gysel, Diane RouzaudDisclosure of Interests:None declared


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1586.2-1586
Author(s):  
M. Greco ◽  
M. J. García de Yébenes ◽  
F. J. Nóvoa Medina ◽  
J. A. Hernandez Beriain ◽  
M. M. Riaño Ruiz ◽  
...  

Background:The presence of anti-aminoacyl transfer RNA synthetase (ARS) autoantibodies is essential for the antisynthetase syndromes (ASSD) diagnosis; as well, Anti-Jo1 ARS positivity is the highest weighted criterion in the 2017 EULAR/ACR criteria for idiopathic inflammatory myopathies (IIM).Previous studies have shown differences in ARS specificity between different blot assays. Nevertheless, an adequate clinical correlation and its detection by another monospecific-assays or indirect immunofluorescence assay (IIFA), can safeguard it.Objectives:To evaluate the prevalence of Anti-Jo1 ARS and their performance in the ASSD and IIM diagnosis criteria fulfillment in a real-world population.Methods:We performed an observational retrospective study in one center in which IIFA and a blot assay (EUROLINE ANA Profile 3-IgG that include Anti-Jo1 but not other ARS) were performed in all cases with autoimmune disease suspicion (03/2007-07/2019).We assessed: 1) Anti-Jo1 ARS prevalence, 2) The rate of Anti-Jo1 positivity in the performed blot assays, 3) The rate of patients with Anti-Jo1 ARS meeting ASSD or IIM criteria, and 4) The rate of true and false positive Anti-Jo1 ARS considering the IIFA and clinical correlation.Results:A total of 419.361 inhabitants are under the center coverage area at the date, a total of 12.711 blot assays were performed during the observation period, and 61 cases presented Anti-Jo1 ARS positivity. The Anti-Jo1 ARS prevalence in the whole studied population was 0.00014, representing 0.04% positivity of the performed blot assays.Of those with Anti-Jo1 positivity:– Only 4 patients (6.6%) met Solomon´s ASSD criteria and 26 (42.6%) met Connors ASSD criteria (less strict), representing the 0.0009% and 0.006% of the whole population respectively.– Five cases (8.2%) presented a possible or probable IIM by Bohan and Peter criteria and 55 cases (90.16%) presented a probable or definitive IIM by EULAR/ACR criteria, representing 0.001% and the 0.013% of the whole population respectively.– The Anti-Jo1 positivity was not confirmed by a monospecific-assay. Nevertheless, 52 cases (85.25%) presented positive IIFA (>1/80): 27 (51.92%) nuclear, 12 (23.08%) cytoplasmic and 12 (25%) with both patterns. A total of 23 cases presented a fine speckled AC-19/AC-20 pattern; representing the 40.98% of all Anti-Jo1 ARS cases.– Only 4 cases (6.56%) did not meet any of the ASSD or IIM classification criteria. And only 4 cases (6.56%) fulfilled Solomon ASSD criteria or presented a probable IIM by Bohan and Peter criteria, or a definite IIM by EULAR/ACR criteria. Thus, we can estimate that 6.56% of the cases were clinically false positives and other 6.56% were clinically true positive; leading a gap of 86.88% of cases with Anti-Jo1 ARS that only fulfill Connors ASSD criteria (less strict) or a not complete score to confirm the IIM diagnosis by Bohan and Peter or EULAR/ACR criteria.Conclusion:The low prevalence of Anti-Jo1 ARS positivity observed and the low number of cases with confirmed ASSD or IIM in the Anti-Jo1 positive detected cases, suggest that:– It is not efficient to test it by screening.– It should be tested only under adequate clinical suspicion.– Probably it is not convenient to include it in not myositis specific blot assays. Despite it is desirable to incorporate the evaluation of myositis specific antibodies in the IIM classification criteria; the differences observed between Bohan and Peter and 2017 EULAR/ACR criteria fulfillment in our series, suggest that the latter could be overweighting the Anti-Jo1 ARS positivity.Disclosure of Interests:Martín Greco: None declared, María Jesús García de Yébenes: None declared, Francisco Javier Nóvoa Medina Speakers bureau: I have been paid as a speaker for a few medical talks, José A Hernandez Beriain: None declared, Marta María Riaño Ruiz: None declared, María Jesús Montesa: None declared, Iñigo Rua-Figueroa: None declared, Estíbaliz Loza Grant/research support from: Roche, Pfizer, Abbvie, MSD, Novartis, Gebro, Adacap, Astellas, BMS, Lylly, Sanofi, Eisai, Leo, Sobi, Loreto Carmona Grant/research support from: Novartis Farmaceutica, SA, Pfizer, S.L.U., Merck Sharp & Dohme España, S.A., Roche Farma, S.A, Sanofi Aventis, AbbVie Spain, S.L.U., and Laboratorios Gebro Pharma, SA (All trhough institution)


2021 ◽  
Vol 8 (1) ◽  
pp. e000458
Author(s):  
Huijing Wang ◽  
Yunjie Gao ◽  
Yanhong Ma ◽  
Fanghao Cai ◽  
Xiaohan Huang ◽  
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ObjectiveTo evaluate the performance of the European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) SLE classification criteria in a cohort of patients with biopsy-confirmed lupus nephritis (LN) and their renal prognosis.MethodsPatients with newly diagnosed SLE attending and followed up for >12 months were included. A retrospective review of all patients with renal biopsy fulfilling a consensus expert opinion during 2014 and 2018. Clinical, serological and pathological data were collected and each patient was assigned a high/low criteria scores (HS/LS) group. Survival curves for flare adjusted for multiplicity on renal flares, was applied to the two groups.ResultsApplying EULAR/ACR criteria in our cohort of 126 patients, 6 (4.76%) did not meet the criterion, resulting in a sensitivity of 95.24%. The EULAR/ACR criteria scores was positively correlated with SLE disease activity index scores. Additionally, we noticed that a significant difference in clinical and immunological manifestations between HS and LS group. We observed a higher proportions of class Ⅲ or Ⅳ LN and lower proportions of class Ⅱ or V LN (p=0.034) and pathological higher activity index in HS group (p=0.007). Compared with LS groups, patients involved more severe renal damage and achieved higher rate of complete remission in the HS group. The Kaplan-Meier exploratory analyses, adjusted for LN classification, estimated glomerular filtration rate, activity index and chronicity index and induction and maintenance treatments, showed that patients in the HS group had a tendency of higher renal flare risk than that in the LS group (HR=0.21, p=0.04).ConclusionsThe EULAR/ACR criteria performed high sensitivity in identifying SLE in this cohort of biopsy-confirmed LN. Patients with LN with high criteria scores had more extrarenal manifestations, and worse renal prognosis in the short and long terms.


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