scholarly journals Comparison of Sensitivities of American College of Rheumatology and Systemic Lupus International Collaborating Clinics Classification Criteria in Childhood-onset Systemic Lupus Erythematosus

2019 ◽  
Vol 46 (7) ◽  
pp. 731-738 ◽  
Author(s):  
Jessie J. Tao ◽  
Linda T. Hiraki ◽  
Deborah M. Levy ◽  
Earl D. Silverman
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Classification Criteria ◽  
Childhood Onset ◽  
Systemic Lupus ◽  
Specific Criterion ◽  
Primary Sjögren Syndrome ◽  
American College Of Rheumatology ◽  
Acr Criteria ◽  
Sick Children

Objective.Currently there are 2 different classification criteria for systemic lupus erythematosus (SLE): American College of Rheumatology (ACR) and Systemic Lupus International Collaborating Clinics (SLICC). The aim of this study was to compare the sensitivities of ACR and SLICC criteria in childhood-onset SLE (cSLE) using a large, multiethnic cohort.Methods.We conducted a retrospective study of 722 patients diagnosed with cSLE at The Hospital for Sick Children (SickKids). Prospectively collected data from SickKids’ Lupus Database were reviewed/validated against medical records prior to ACR and SLICC scoring based on cumulative symptoms up to the last visit. Sensitivities were compared using McNemar’s test. Descriptive statistics were used to identify SLE features unique to each set of criteria and autoantibodies not included in either.Results.ACR and SLICC sensitivities were as follows: 92.4% and 96.3% overall (p = 0.001); 82.5% and 91.3% (p = 0.01) in those scored ≤ 1 year from diagnosis; 92.7% and 97.9% (p = 0.02) in those scored 2–3 years from diagnosis. Forty-eight of 55 (87.3%) patients who did not meet ACR criteria met SLICC criteria through SLICC-specific criterion or renal biopsy. Twenty of 27 (74.1%) patients who did not meet SLICC criteria met ACR criteria as a result of photosensitivity (73.9%) and ACR lymphopenia criteria (26.1%). Six of 7 patients (85.7%) who were clinically diagnosed with cSLE but did not meet either SLICC or ACR criteria had anti-Ro antibodies.Conclusion.SLICC criteria were significantly more sensitive than ACR criteria in cSLE classification, especially early in the disease course. Because of the extreme rarity of primary Sjögren syndrome in children, one may consider adding anti-Ro antibodies to the classification criteria for cSLE because they are present in ∼40% of patents with cSLE.

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.

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

European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) SLE classification criteria item performance

2021 ◽  
pp. annrheumdis-2020-219373
Author(s):  
Martin Aringer ◽  
Ralph Brinks ◽  
Thomas Dörner ◽  
David Daikh ◽  
Marta Mosca ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Metabolic Diseases ◽  
Classification Criteria ◽  
Joint Involvement ◽  
Systemic Lupus ◽  
Entry Criterion ◽  
American College Of Rheumatology ◽  
European League Against Rheumatism ◽  
European League

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.

Use of Consensus Methodology to Determine Candidate Items for Systemic Lupus Erythematosus Classification Criteria

2018 ◽  
Vol 46 (7) ◽  
pp. 721-726 ◽  
Author(s):  
Sindhu R. Johnson ◽  
Dinesh Khanna ◽  
David Daikh ◽  
Ricard Cervera ◽  
Nathalie Costedoat-Chalumeau ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Clinical Care ◽  
Nominal Group Technique ◽  
Steering Committee ◽  
Classification Criteria ◽  
Nominal Group ◽  
Systemic Lupus ◽  
High Performing ◽  
American College Of Rheumatology

Objective.Given the complexity and heterogeneity of systemic lupus erythematosus (SLE), high-performing classification criteria are critical to advancing research and clinical care. A collaborative effort by the European League Against Rheumatism and the American College of Rheumatology was undertaken to generate candidate criteria, and then to reduce them to a smaller set. The objective of the current study was to select a set of criteria that maximizes the likelihood of accurate classification of SLE, particularly early disease.Methods.An independent panel of international SLE experts and the SLE classification criteria steering committee (conducting SLE research in Canada, Mexico, United States, Austria, Germany, Greece, France, Italy, and Spain) ranked 43 candidate criteria. A consensus meeting using nominal group technique (NGT) was conducted to reduce the list of criteria for consideration.Results.The expert panel NGT exercise reduced the candidate criteria for SLE classification from 43 to 21. The panel distinguished potential “entry criteria,” which would be required for classification, from potential “additive criteria.” Potential entry criteria were antinuclear antibody (ANA) ≥ 1:80 (HEp-2 immunofluorescence), and low C3 and/or low C4. The use of low complement as an entry criterion was considered potentially useful in cases with negative ANA. Potential additive criteria included lupus nephritis by renal biopsy, autoantibodies, cytopenias, acute and chronic cutaneous lupus, alopecia, arthritis, serositis, oral mucosal lesions, central nervous system manifestations, and fever.Conclusion.The NGT exercise resulted in 21 candidate SLE classification criteria. The next phases of SLE classification criteria development will require refinement of criteria definitions, evaluation of the ability to cluster criteria into domains, and evaluation of weighting of criteria.

scholarly journals PARTICULARITIES OF PEDIATRIC SYSTEMIC LUPUS ERYTHEMATOSUS – LITERATURE REVIEW

2017 ◽  
Vol 26 (1) ◽  
pp. 5-7
Author(s):  
Oana-Maria Farkas ◽  
◽  
Sigrid Covaci ◽  
Alexis-Virgil Cochino ◽  
◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Pediatric Population ◽  
Signs And Symptoms ◽  
Classification Criteria ◽  
Neurological Involvement ◽  
Systemic Lupus ◽  
Pediatric Systemic Lupus Erythematosus ◽  
American College Of Rheumatology ◽  
Clinical Onset

Pediatric Systemic Lupus Erythematosus (pSLE) is a complex autoimmune disease with onset of symptoms before 18 years of age, accounting for 18-20% of all SLE cases. Although the American College of Rheumatology (ACR) classification criteria and the SLICC (Systemic Lupus International Collaborating Clinics) classification criteria for adults with SLE are commonly applied to pSLE, its clinical onset is different. Renal and neurological involvement tend to be more common and more severe in pediatric population as compared to adults, being therefore major determinants of prognosis and mortality. Renal biopsy should be performed as early as possible in every case of pSLE with signs and symptoms of renal impairment.

A comparison and review of three sets of classification criteria for systemic lupus erythematosus for distinguishing systemic lupus erythematosus from pure mucocutaneous manifestations in the lupus disease spectrum

Lupus ◽  
2020 ◽  
Vol 29 (14) ◽  
pp. 1854-1865
Author(s):  
Hui Jin ◽  
Tao Huang ◽  
Ruifang Wu ◽  
Ming Zhao ◽  
Haijing Wu ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Diagnostic Criteria ◽  
Lupus Erythematosus ◽  
Sample Selection ◽  
Cutaneous Lupus Erythematosus ◽  
Classification Criteria ◽  
Systemic Lupus ◽  
American College Of Rheumatology ◽  
Disease Spectrum ◽  
European League Against Rheumatism

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.

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

2019 ◽  
Vol 78 (9) ◽  
pp. 1151-1159 ◽  
Author(s):  
Martin Aringer ◽  
Karen Costenbader ◽  
David Daikh ◽  
Ralph Brinks ◽  
Marta Mosca ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Validation Cohort ◽  
Classification Criteria ◽  
Systemic Lupus ◽  
New Classification ◽  
Entry Criterion ◽  
American College Of Rheumatology ◽  
European League Against Rheumatism ◽  
European League

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.

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