Abstract

Importance/backgroundSeveral diagnostic criteria have been developed to effectively diagnose systemic lupus erythematosus (SLE). Three criteria are most common, namely the American College of Rheumatology (ACR)-1997, the Systemic Lupus International Collaborating Clinics (SLICC)-2012, and the European League Against Rheumatism (EULAR/ACR)-2019. Whether they also apply to juvenile SLE is unclear. ObjectiveTo examine the diagnostic accuracy of ACR-1997, SLICC-2012, and EULAR/ACR-2019 for juvenile SLE. Data sourcesA comprehensive search of PubMed, Cochrane, and Embase was conducted up to 26 March 2022. Study selectionWe included all study designs in which patients had any index tests for ACR-1997, SLICC-2012, or EULAR/ACR-2019; both full-text papers and conference abstracts published in English were used. Exclusion criteria were as follows: (1) case reports; (2) adult subjects; or (3) did not report sufficient information to acquire true positive, false positive, true negative, and false negative values of diagnostic criteria. Data extraction and synthesisTwo authors independently screened studies, extracted relevant data, and assessed the risk of bias. Main outcomes and measuresFirst, a meta-analysis of the diagnostic accuracy of EULAR/ACR-2019 and a hierarchical summary receiver operating characteristic (HSROC) model was performed to estimate sensitivity and specificity with 95% confidence intervals (CIs). We then carried out a network meta-analysis to compare the performances of these three diagnostic criteria. ResultsIn total, 17 relevant studies that included 2339 juvenile SLE patients were eligible to analyze pooled accuracy. In the meta-analysis, 10 studies (1613 cases) reported the diagnostic performance of EULAR/ACR-2019, showing a pooled sensitivity of 0.92 (95% CI, 0.89–0.95), pooled specificity of 0.89 (0.77–0.95), and area under HSROC of 0.96 (0.94–0.97). In the network meta-analysis, the SLICC-2012 (0.94, 0.92–0.96) had the highest sensitivity, followed by EULAR/ACR-2019 (0.93, 0.90–0.95), and ACR-1997 (0.78, 0.72–0.82); the ACR-1997 (0.96, 0.92–0.98) demonstrated the highest specificity. EULAR/ACR-2019 (0.92, 0.87–0.96) and SLICC-2012 (0.92, 0.86–0.96) had the similar specificity. Conclusions and relevanceWe found that the applicability of the new EULAR/ACR-2019 criteria in juvenile SLE is not yet the best diagnostic tool. Trial registrationPROSPERO CRD42022321514.

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