scholarly journals P140 Validation of EULAR/ACR 2019 classification criteria for SLE in a south Indian juvenile SLE cohort

Rheumatology ◽  
2020 ◽  
Vol 59 (Supplement_2) ◽  
Author(s):  
Harikrishnan Bhaskaran Nair ◽  
Suma Balan

Abstract Background To date there is no specific classification criteria for juvenile systemic lupus erythematosus (jSLE). Our aim is to compare the real world performance of EULAR/ACR 2019 criteria when applied to a known cohort of jSLE cases in Kerala, India. Methods We retrospectively reviewed the electronic medical record of all patients (n = 735), admitted under the Pediatric Rheumatology Department, Amrita Institute of Medical Sciences, Kochi, a tertiary care center in Kerala, India, during the period January 2012 to June 2019. 107 patients were diagnosed with jSLE by a qualified experienced pediatric rheumatologist and were independently assessed by an adult rheumatologist. Every sixth patient of the remaining 628 patients were selected as the control (n = 105). Patients were excluded if the diagnosis was uncertain or, if there was an overlap with jSLE. Each patient was evaluated to see if he or she satisfied the ACR-1997, SLICC-2012, or EULAR/ACR- 2019 criteria. Results Female: male ratio in cases and controls was 4.1:1 and 2.4 :1 respectively. Mean age for cases and controls was 12.6 +/-2.9 and 11.8 +/-3.5 respectively. The comparison of performance of each criterion is shown in table 1. Table 1: Validation of the EULAR/ACR 2019 SLE classification criteria using clinically diagnosed 107 jSLE cases. ACR- American College of Rheumatology; EULAR- European League Against Rheumatism; SLICC-Systemic Lupus International Collaborating Clinics; PPV- positive predictive value; NPV- negative predictive value Conclusion The new EULAR/ACR 2019 criteria achieved sensitivity, specificity, PPV, NPV and accuracy greater than SLICC 2012 criteria. It also achieved sensitivity, NPV and accuracy greater than 1997 ACR criteria. Disclosures H. Bhaskaran Nair None. S. Balan None.

2021 ◽  
pp. annrheumdis-2020-219373
Author(s):  
Martin Aringer ◽  
Ralph Brinks ◽  
Thomas Dörner ◽  
David Daikh ◽  
Marta Mosca ◽  
...  

Background/objectivesThe 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.MethodsWe 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.ResultsPositive 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.ConclusionsChanging 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.


Lupus ◽  
2020 ◽  
Vol 29 (14) ◽  
pp. 1854-1865
Author(s):  
Hui Jin ◽  
Tao Huang ◽  
Ruifang Wu ◽  
Ming Zhao ◽  
Haijing Wu ◽  
...  

Although the original purpose of the systemic lupus erythematosus (SLE) classification criteria was to distinguish SLE from other mimic diseases, and to facilitate sample selection in scientific research, they have become widely used as diagnostic criteria in clinical situations. It is not known yet if regarding classification criteria as diagnostic criteria, what problems might be encountered? This is the first study comparing the three sets of classification criteria for SLE, the 1997 American College of Rheumatology (ACR’97), 2012 Systemic Lupus International Collaborating Clinics (SLICC’12) and 2019 European League Against Rheumatism/American College of Rheumatology (EULAR/ACR’19), for their ability to distinguish patients with SLE from patients with pure mucocutaneous manifestations (isolated cutaneous lupus erythematosus without internal disease, i-CLE) in the lupus disease spectrum. 1,865 patients with SLE and 232 patients with i-CLE were recruited from a multicenter study. We found that, due to low specificity, none of the three criteria are adept at distinguishing patients with SLE from patients with i-CLE. SLICC’12 performed best among the original three criteria, but if a positive ANA was removed as an entry criterion, EULAR/ACR’19 would performed better. A review of previous studies that compared the three sets of criteria was presented in this work.


2019 ◽  
Vol 78 (9) ◽  
pp. 1151-1159 ◽  
Author(s):  
Martin Aringer ◽  
Karen Costenbader ◽  
David Daikh ◽  
Ralph Brinks ◽  
Marta Mosca ◽  
...  

ObjectiveTo 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).MethodsThis 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.ResultsThe 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.ConclusionThese 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.


2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1177.2-1178
Author(s):  
T. Reshetnyak ◽  
A. Shumilova ◽  
S. Glukhova ◽  
F. Cheldieva ◽  
T. Lisitsyna ◽  
...  

Background:Classification criteria for SLE need validation and selection of the most advanced ones for clinical practiceObjectives:To validate Systemic Lupus International Collaborating Clinics (SLICC)-12, American College of Rheumatology (ACR)-97 and 2019 European League against Rheumatism/American College of Rheumatology (EULAR/ACR-2019) for SystemicLupus Erythematosus classification criteria on a patient cohort from V.A. Nasonova Scientific Research Institute of Rheumatology (Moscow)Methods:This prospective study included 252 (n=152 with SLE and n=100 non SLE: 74 – with systemic sclerosis (SC) and 26 – with primary antiphospholipid syndrome (pAPS)) patients who were sequentially admitted to the 4th Rheumatology Department from December 2018 to June 2020. All of the patient records were reevaluated by expert rheumatologist in order to determine if they agree with the diagnosis. For every patient, a check-list of SLE-related features was filled out. The association between clinical diagnosis and diagnosis generated on the basis of ACR-97, SLICC-12 and EULAR/ACR-2019classification criteria was assessed. The overall sensitivity and specificity of ACR-97, SLICC-12 and EULAR/ACR-2019 classifications, as well as the sensitivity and specificity according to disease duration was calculated. The predictive value of every criterion in ACR-97, SLICC-12 and 2019 EULAR/ACR classification was assessed using logistic regression analysis and receiver operating characteristic (ROC) curves.Results:According to ACR-97 criteria, reliable SLE was diagnosed in 131 (86%), according to SLICC-2012 – in 145 (95.3%) and according to EULAR/ACR-2019 – in 144 (94.7%) of 152 patients, respectively. We identified the criteria that were significantly more represented in the SLE-group. The sensitivity and specificity (Table 1) did not change depending on the ANA titers (1/160 and 1/320) and the duration of the disease.Table 1.The sensitivity and specificity of SLE criteria in 152 SLE patients and 100 non SLE patientsSLE classification criteriaAUC, 95% CICut pointSensivitySpecifityACR-970.654 (0.550-0.758)489.736SLICC-120.616 (0.505-0.728)493.1432019 EULAR/ACR0.609 (0.492-0.727)10*97.448Note. ACR-97 – American College of Rheumatology classification from 1997; SlICC-12 – Systemic lupus International Collaboration Clinics classification from 2012; 2019 European League Against Rheumatism/American College of Rheumatology. ANA – anti-nuclear antibody more than 1/320 n=116; ANA < 320 n= 36. AUC – area under the ROC curve; Cut point - reference point for the number of criteria, * number of points ≥ 10.When excluding patients with SC in a single-factor logistic model of patients with SLE and pAPS, indicating the number of criteria, the sensitivity was for ACR-97 - 86, for SlICC-12 - 95, for 2019 EULAR/ACR - 95, the specificity was 100, 62 and 62, respectively.Conclusion:Evaluation of the criteria by level ANA revealed the highest sensitivity for 2019 EULAR/ACR and SLICC-12, the specificity was low for all three criteria. In the analysis of patients with SLE and pAPS, the sensitivity was highest for 2019 EULAR/ACR and SLICC-12, the specificity was 100 for ACR-97.Disclosure of Interests:None declared


2020 ◽  
Vol 95 (3) ◽  
pp. 151-161
Author(s):  
Yeon-Ah Lee

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.


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