The performances of the ACR 1997, SLICC 2012, and EULAR/ACR 2019 classification criteria in pediatric systemic lupus erythematosus

2020 ◽  
pp. jrheum.200871
Author(s):  
Ezgi Deniz Batu ◽  
Ummusen Kaya Akca ◽  
Aysenur Pac Kısaarslan ◽  
Erdal Sağ ◽  
Ferhat Demir ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Antibody Test ◽  
Classification Criteria ◽  
Joint Involvement ◽  
Systemic Lupus ◽  
Pediatric Systemic Lupus Erythematosus ◽  
American College Of Rheumatology ◽  
Hematological Manifestations ◽  
Pediatric Sle

Objective Systemic lupus erythematosus (SLE) is a chronic autoimmune disease. The ACR (American College of Rheumatology) 1997, SLICC (Systemic Lupus International Collaborating Clinics) 2012, and EULAR (European League Against Rheumatism)/ACR 2019 SLE classification criteria are formed based on data mainly from adult patients. We aimed to test the performances of the SLE classification criteria among pediatric SLE patients. Methods Pediatric SLE patients (n=262; 80.9% female) were included from three different centers in Turkey. As controls, 174 children (60.9% female) with other diseases who had ANA (antinuclear antibody) test results were included. The gold standard for SLE diagnosis was expert opinion. Results The sensitivities of the ACR 1997, SLICC 2012, and EULAR/ACR 2019 criteria were 68.7%, 95.4%, and 91.6%, respectively. The specificities of the ACR 1997, SLICC 2012, and EULAR/ACR 2019 criteria were 94.8%, 89.7%, and 88.5%, respectively. 18 SLE patients met the SLICC 2012 but not the EULAR/ACR 2019 criteria. Among these, hematologic involvement was prominent (13/18; 72.2%). Eight SLE patients fulfilled the EULAR/ACR 2019 but not the SLICC 2012 criteria. Among these, joint involvement was prominent (6/8; 75%). Conclusion This is the largest cohort study of pediatric SLE testing the performances of all three classification criteria. The SLICC 2012 criteria yielded the best sensitivity, while the ACR 1997 criteria had the best specificity. SLICC 2012 criteria performed better than EULAR/ACR 2019 criteria. Separation of different hematological manifestations in the SLICC 2012 criteria might have contributed to the higher performance of this criteria set.

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.

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.

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.

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.

scholarly journals Citrulline Dependence of Anti-Cyclic Citrullinated Peptide Antibodies in Systemic Lupus Erythematosus as a Marker of Deforming/Erosive Arthritis

2009 ◽  
Vol 36 (12) ◽  
pp. 2682-2690 ◽  
Author(s):  
PRASANTHI KAKUMANU ◽  
ERIC S. SOBEL ◽  
SONALI NARAIN ◽  
YI LI ◽  
JUN AKAOGI ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Joint Involvement ◽  
Healthy Controls ◽  
Systemic Lupus ◽  
Cyclic Citrullinated Peptide ◽  
Erosive Arthritis ◽  
American College Of Rheumatology ◽  
Citrullinated Peptide ◽  
Peptide Antibodies

Objective.Anti-cyclic citrullinated peptide (CCP) antibodies are a serological marker for rheumatoid arthritis (RA); up to 10%–15% of patients with systemic lupus erythematosus (SLE) are also positive. While anti-CCP in RA is citrulline-dependent, anti-CCP in some other diseases is citrulline-independent and reacts with both CCP and the unmodified (arginine-containing) cyclic arginine peptide (CAP). We investigated the citrulline dependence of anti-CCP and its significance in the arthritis of SLE.Methods.IgG anti-CCP was compared by ELISA to anti-CAP in sera from patients with SLE (n = 335) and RA (n = 47) and healthy controls (n = 35). SLE patients were divided into 5 groups based on their joint involvement: subset I: deforming/erosive arthritis (n = 20); II: arthritis fulfilling (or likely fulfilling) American College of Rheumatology criteria for RA but without erosions (n = 18); III: joint swelling but not fulfilling RA criteria (n = 39); IV: arthritis without documented joint swelling (n = 194); and V: no arthritis (n = 58).Results.Anti-CCP (> 1.7 units) was found in 68% (32/47) of patients with RA and 17% (55/329) of those with SLE. It was more common in SLE patients with deforming/erosive arthritis (38%). High anti-CCP (> 10 units) was found in RA (26%) and deforming/erosive SLE (12%). High anti-CCP/CAP ratios (> 2, indicating a selectivity to CCP) were found in 91% of anti-CCP-positive RA and 50% of anti-CCP-positive SLE patients with deforming/erosive arthritis. Patients from subset II did not have high anti-CCP/CAP.Conclusion.Citrulline dependence or high levels (> 10) of anti-CCP were common in SLE patients with deforming/erosive arthritis, while most anti-CCP in SLE patients was citrulline-independent. This may be useful in identifying a subset of SLE patients with high risk for development of deforming/erosive arthritis.

scholarly journals MAGE-B2 Autoantibody: A New Biomarker for Pediatric Systemic Lupus Erythematosus

2008 ◽  
Vol 35 (12) ◽  
pp. 2430-2438 ◽  
Author(s):  
ALICE D.C. HOFTMAN ◽  
LEI-QIAN TAI ◽  
SHEILA TZE ◽  
DAVID SELIGSON ◽  
RICHARD A. GATTI ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Disease Activity ◽  
Lupus Erythematosus ◽  
Activity Index ◽  
Disease Activity Index ◽  
Systemic Lupus ◽  
Western Blots ◽  
Pediatric Systemic Lupus Erythematosus ◽  
Embryonic Antigen ◽  
Pediatric Sle

ObjectiveMelanoma-associated antigen gene B2 (MAGE-B2) encodes an embryonic antigen normally silenced after birth except in testis and placenta. We identified the MAGE-B2 gene and autoantibodies in pediatric patients with systemic lupus erythematosus (SLE) glomerulonephritis. We investigated the prevalence of MAGE-B2 autoantibodies in association with active SLE, to determine a pathogenetic role of MAGE-B2 protein through its distribution in cells and tissues.MethodsAcross-sectional study analyzed the frequency of MAGE-B2 autoantibodies in 40 patients with pediatric SLE, 23 adult controls, and 16 patients with pediatric juvenile rheumatoid arthritis (JRA) using Western blots containing recombinant MAGE-B2. SLE Disease Activity Index 2000 (SLEDAI-2K) and British Isles Lupus Assessment Group (BILAG) index measured SLE disease activity. Tissue distribution of MAGE-B2 protein was assessed by immunohistochemistry, immunofluorescence, and Western blots.ResultsSeventeen (43%) of 40 pediatric SLE patients had MAGE-B2 autoantibodies as compared to 0 of 16 JRA patients and 2 of 23 adult controls. SLE disease activity was significantly higher in MAGE-B2 autoantibody-positive versus autoantibody-negative patients (SLEDAI-2K, mean 10.9 vs 5.2, p = 0.013; BILAG, mean 15.3 vs 6.3, p = 0.023). Active nephritis was more prevalent (60% vs 24%) inMAGE-B2 autoantibody-positive than autoantibody-negative SLE patients. MAGE-B2 protein was visualized in SLE kidney proximal convoluted tubules and in tumor epithelial cells, but not in lymphoblastoid cells.ConclusionMAGE-B2 autoantibody appears to be a clinically relevant biomarker for pediatric SLE disease activity and nephritis.

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