Limited sensitivity and specificity of the ACR/EULAR-2019 classification criteria for SLE in JSLE?—observations from the UK JSLE Cohort Study

Abstract

Objectives This study aimed to test the performance of the “new” American College of Rheumatology (ACR) and European League Against Rheumatism (EULAR) criteria, that include anti-nuclear antibodies (ANA) positivity as entry criterion, in juvenile-onset systemic lupus erythematosus (jSLE). Methods Performance of the ACR/EULAR-2019 criteria were compared with Systemic Lupus International Collaborating Clinics (SLICC-2012), using data from children and young people (CYP) in the UK JSLE Cohort Study (n = 482), with the ACR-1997 criteria used as reference standard. An “unselected” cohort of CYP positive for ANA (n = 129) was used to calculate positive/negative predictive values of the criteria. Results At both first and last visit, the number of patients fulfilling the different classification criteria varied significantly (p < 0.001). Sensitivity of SLICC-2012 criteria was higher when compared to ACR/EULAR-2019 at first and last visit (98% vs 94%, first visit, and 98% vs 96%, last visit; p < 0.001), when all available CYP were considered. ACR/EULAR-2019 criteria were more specific when compared to SLICC-2012 (77% vs 67%, first visit, and 81% vs 71%, last visit; p < 0.001). Significant differences between the classification criteria were mainly caused by the variation in ANA positivity across ages. In the “unselected” cohort of ANA positive CYP, ACR/EULAR-2019 criteria produced the highest false positive classification (6/129, 5%) . Conclusion In CYP, ACR/EULAR-2019 criteria are not superior to SLICC-2012 or ACR-1997 criteria. If classification criteria are designed to include CYP and adult populations, paediatric rheumatologists should be included in the consensus and evaluation process, as seemingly minor changes can significantly affect outcomes.

Performance of 2019 EULAR/ACR Classification Criteria for Systemic Lupus Erythematosus in Children and Adults : A Retrospective Observational Study

“…The EULAR/ACR-2019 criteria may miss a signi cant proportion of jSLE patients, mostly because of the absence of ANA positivity. On the other hand, positive ANA can be found in a variety of autoimmune diseases, which may result in misclassi cation as SLE based on the new criteria [14]. Within ANA-positive jSLE patients, the sensitivity of the EULAR/ACR criteria was higher (99.5%), but the speci city dropped to 75.9%, signi cantly lower than the results (98.4%) of Ma et al [12].…”

Comparison of the sensitivity of the EULAR / ACR 2019 and SLICC 2012 classification criteria in a Colombian population with systemic lupus erythematosus

“…However, they stated that the performance of the 2019 criteria in the youth population can be improved if they are classified using 13 points and not 10 as originally proposed. Other recent studies do not find differences between the three criteria (EULAR/ACR 2019, SLICC 2012, and ACR 97) [ 31 ]. However, this contrasts with data published by Levinsky et al [ 28 ] who documented a better sensitivity of the EULAR/ACR 2019 with respect to the SLICC 2012 in a population with juvenile SLE.…”

The spectrum of renal diseases with lupus-like features: a single-center study

“…Smith et al. [ 12 ] found that a subset of patients presenting with organ damage including biopsy of class III/IV was ANA-negative with half eventually developing ANA-positivity. The authors speculate that ANA-negativity, particularly in young SLE patients, may be related to genetic factors that contribute to the inflammation and tissue damage in the absence of autoantibodies.…”