Does Leukopenia Influence Performance of the New European League Against Rheumatism/American College of Rheumatology Classification Criteria in an African‐Descendent Population With Childhood‐Onset Systemic Lupus Erythematosus? Comment on the Article by Aringer et al

2020 ◽  
Vol 72 (4) ◽  
pp. 694-695
Author(s):  
Adriana R. Fonseca ◽  
Marta C. F. Rodrigues ◽  
Flavio Sztajnbok ◽  
Marcelo G. P. Land ◽  
Sheila K. F. Oliveira
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Classification Criteria ◽  
Childhood Onset ◽  
Systemic Lupus ◽  
American College Of Rheumatology ◽  
European League Against Rheumatism ◽  
European League ◽  
Onset Systemic Lupus Erythematosus

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 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 POS1295 PERFORMANCE OF 2019 EULAR/ACR CLASSIFICATION CRITERIA FOR SYSTEMIC LUPUS ERYTHEMATOSUS IN A PEDIATRIC POPULATION – A MULTICENTER STUDY

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 929.1-929
Author(s):  
Y. Levinsky ◽  
M. Broide ◽  
S. Kagan ◽  
O. Goldberg ◽  
O. Scheuerman ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Pediatric Patients ◽  
Classification Criteria ◽  
Systemic Lupus ◽  
Juvenile Onset ◽  
American College Of Rheumatology ◽  
European League Against Rheumatism ◽  
Onset Systemic Lupus Erythematosus

Background:The “European League Against Rheumatism” and “American College of Rheumatology” 2019 (EULAR/ACR-19) criteria for the diagnosis of Systemic Lupus Erythematosus (SLE) were recently published, with the stated goal of maintaining the level of sensitivity and raising the level of specificity for classification of SLE in adults.Objectives:We aimed to examine the function of the new EULAR/ACR-19 criteria in a population of children and compare them to the SLICC-12 and ACR-97 criteria.Methods:In this multicenter study the charts of jSLE patients from three tertiary medical centers were reviewed and compared to patients with non-jSLE diagnosis. Pediatric rheumatologists, blinded to the original diagnosis, reviewed and diagnosed all cases. Pediatric patients’ clinical and laboratory data were retrospectively extracted and then examined with regard to how they met the new and old criteria.Results:Included were 225 patients (112 jSLE, 113 non-SLE). When applied to juvenile SLE classification, the sensitivity of the new EULAR/ACR-19 criteria was 0.96 (0.9-.0.99) and the specificity was 0.89 (0.82-0.94). These were comparable to the Systemic Lupus International Collaborating Clinics (SLICC) criteria. The sensitivity of the EULAR/ACR-19 criteria improves over time and was 0.83 twelve months following disease onset, reaching 0.96 after longer than 24 months.Conclusion:Among a cohort of jSLE patients, sensitivity of the new EULAR/ACR-19 criteria was found to be high and specificity may have improved slightly compared to the SLICC-12 criteria. We support the use of the new classification criteria for pediatric patients in future jSLE studies, but it should be noted that its specificity is lower than for adults.ACR-97SLICC-12EULAR/ACR-19Sensitivity (95% CI)0.79 (0.70-0.86)0.96 (0.9-0.99)0.96 (0.9-.0.99)Specificity (95% CI)0.94 (0.88-0.97)0.85 (0.77-0.91)0.89 (0.82-0.94)Accuracy (95% CI)0.86 (0.81-0.9)0.9 (0.86-0.94)0.92 (0.88-.0.96)Positive Likelihood Ratio (95% CI)12.7(6.1-26.2)6.35(4.1-9.9)9.0 (5.3-15.4)Negative Likelihood Ratio (95% CI)0.23(0.16-0.33)0.05(0.02-0.12)0.05(0.02-0.12)Diagnostic odds ratio (95% CI)55.5(22.80-135.0)120.85(43.0-340.0)180.1(61.3-529.4)References:[1]Smith EMD, Lythgoe H, Midgley A, Beresford MW, Hedrich CM. Juvenile-onset systemic lupus erythematosus: Update on clinical presentation, pathophysiology and treatment options. Clin Immunol 2019 published on December 2019. doi: 10.1016/j.clim.2019.108274[2]Massias JS, Smith EMD, Al-Abadi E, Armon K, Bailey K, Ciurtin C, et al. Clinical and laboratory characteristics in juvenile-onset systemic lupus erythematosus across age groups. Lupus 2020;29(5):474–81.[3]Hochberg MC. Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum. 1997 Sep;40(9):1725[4]Petri M, Orbai AM, Alarcõn GS, Gordon C, Merrill JT, Fortin PR, et al. Derivation and validation of the systemic lupus international collaborating clinics classification criteria for systemic lupus erythematosus. Arthritis Rheum 2012 Aug;64(8):2677-86[5]Hartman EAR, van Royen-Kerkhof A, Jacobs JWG, Welsing PMJ, Fritsch-Stork RDE. Performance of the 2012 Systemic Lupus International Collaborating Clinics classification criteria versus the 1997 American College of Rheumatology classification criteria in adult and juvenile systemic lupus erythematosus. A systematic review and meta-an. Autoimmun Rev. 2018;17(3):316–22.[6]Aringer M, Costenbader K, Daikh D, Brinks R, Mosca M, Ramsey-Goldman R, et al. 2019 European League Against Rheumatism/American College of Rheumatology Classification Criteria for Systemic Lupus Erythematosus. Arthritis Rheumatol 2019;71(9):1400–12.Disclosure of Interests:None declared

Sign in / Sign up

Export Citation Format

Share Document