Abstract
ObjectivesThis study aimed to test the performance of the new ACR and EULAR criteria, that include ANA positivity as entry criterion, in JSLE.MethodsPerformance 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.ResultsAt both first and last visits, the number of patients fulfilling the different classification criteria varied significantly (P < 0.001). The sensitivity of the SLICC-2012 criteria was higher when compared with that of the ACR/EULAR-2019 criteria at first and last visits (98% vs 94% for first visit, and 98% vs 96% for last visit; P < 0.001), when all available CYP were considered. The ACR/EULAR-2019 criteria were more specific when compared with the SLICC-2012 criteria (77% vs 67% for first visit, and 81% vs 71% for 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, the ACR/EULAR-2019 criteria produced the highest false-positive classification (6/129, 5%).ConclusionIn CYP, the ACR/EULAR-2019 criteria are not superior to those of the 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.
Highlights
JSLE is a severe, multisystem autoimmune/inflammatory disease characterized by systemic inflammation, tissue and organ damage, and the presence of autoantibodies directed against nuclear auto-antigens [1]
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 1⁄4 482), with the ACR-1997 criteria used as reference standard
The UK JSLE Cohort Study [13] patients included in this study fulfilled the following inclusion criteria: (1) had data collected between July 2016 and January 2019, (2) an ACR-1997 score of 2 at inclusion, and (3) aged
Summary
JSLE is a severe, multisystem autoimmune/inflammatory disease characterized by systemic inflammation, tissue and organ damage, and the presence of autoantibodies directed against nuclear auto-antigens [1]. Accepted and used criteria for SLE were developed by the ACR in 1982 [3], and updated in 1997 (ACR-1997). Those criteria include 11 clinical and laboratory items, 4 being required for classification as SLE. Due to concerns that the ACR criteria may miss some SLE patients, in particular those with LN and autoantibody positivity but limited systemic involvement, the Systemic Lupus International Collaborating Clinics (SLICC-2012) group established further criteria, including 11 clinical and 6 immunological items [5]. SLICC-2012 stipulated that patients with LN and ANA and/or anti-dsDNA antibody positivity can be defined as SLE, in the absence of other clinical criteria [5]. One of these studies included a control group, and was able to assess the specificity: they demonstrated lower specificity for SLICC-2012 (85.3%) when compared with the ACR-1997 criteria (93.4%) [8]
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