Diagnostic accuracy of the American College of Rheumatology-1997, the Systemic Lupus International Collaborating Clinics-2012, and the European League Against Rheumatism-2019 criteria for juvenile systemic lupus erythematosus: A systematic review and network meta-analysis

Abstract

Importance/background: Several 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.

Objective: To examine the diagnostic accuracy of ACR-1997, SLICC-2012, and EULAR/ACR-2019 for juvenile SLE.

Data sources: A comprehensive search of PubMed, Cochrane, and Embase was conducted up to 26 March 2022.

Study selection: We 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 synthesis: Two authors independently screened studies, extracted relevant data, and assessed the risk of bias.

Main outcomes and measures: First, 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.

Results: In 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 relevance: We found that the applicability of the new EULAR/ACR-2019 criteria in juvenile SLE is not yet the best diagnostic tool.

Trial registration: PROSPERO CRD42022321514.

Keywords: Childhood-onset; Diagnosis; Juvenile; Meta-analysis; Pediatric; SLE.