Background/Purpose:

To avoid misclassifications, a new set of classification criteria have been developed by the collaboration of the EULAR/ACR and the draft was presented at the 2017 ACR/ARHP Annual Meeting. To compare the sensitivity and specificity of the new EULAR/ACR criteria with those of the 1997 ACR criteria and 2012 SLICC criteria in juvenile-onset SLE patients.

Methods:

Juvenile SLE patients initially were evaluated by ACR-1997, SLICC-2012 and EULAR/ACR classification criteria at baseline. The diagnostic sensitivity of the three sets of classification criteria were further tested within 1 year of diagnosis and at last patient visit, longitudinally. Subjects with a clinical diagnosis other than SLE for at least 1 year-period, consecutively enrolled as controls.

Results:

A total of 104 juvenile-onset SLE patients were enrolled for the sensitivity performance of classification criteria at diagnosis and 104 controls (69 juvenile idiopathic arthritis, 9 juvenile systemic sclerosis, 5 juvenile dermatomyositis, 1 mixed connective tissue disease, 15 vasculitis and 5 other diseases) for specificity at their last visit. Since the follow-up period was less than 1 year, 12 SLE subjects excluded after baseline evaluation. Finally, 92 SLE subjects were eligible for sensitivity evaluation within 1 year of diagnosis and at last visit. The median age of the SLE patients at diagnosis of clinician was 13.0 years (range 3.1–17.9 years, interquartile range (IQR) 11.1–16.5 years) with median disease duration of 5.0 years (IQR 3.0-8.0 years). The female-to-male ratio was 4.7:1. The newly developed EULAR/ACR classification criteria were more sensitive than SLICC-2012 and ACR-1997 at diagnosis (93.3% versus 91.3% and 85.6%, respectively), and at first year (95.7% versus 94.6% and 90.2%, respectively (p>0.05). At last visit the sensitivity of the new set of criteria and SLICC-2012 were same (97.8 %), but higher compared to ACR-1997 criteria (95.7%). Specificity of the EULAR/ACR criteria (86.5%, n=90) were found to be higher than SLICC-2012 (81.7%, n=85). Compared to SLICC-2012, an additional 5 subjects among 104 controls without SLE succeeded to get rid of misclassification as SLE by the newly developed criteria. However, the performance of the new EULAR/ACR criteria in diagnostic specificity (86.5%), could not reach the level of ACR-1997 criteria (89.4%).

Conclusion:

Juvenile-onset systemic lupus erythematosus was classified by the newly proposed EULAR/ACR criteria with higher sensitivity compared with SLICC-2012 and ACR-1997 at disease onset and within one year of diagnosis. However, last visit assessment demonstrated equal sensitivity between new EULAR/ACR criteria and SLICC-2012. Although the difference was not significant, the new set of criteria seems to be capable of recruiting more children with juvenile SLE to clinical trials. The performance of the newly developed criteria seems more successful in specificity compared to the SLICC-2012. Application of the newly developed EULAR/ACR criteria to the juvenile-onset SLE patients resulted in higher sensitivity and specificity compared to SLICC-2012.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/the-performance-of-the-newly-proposed-eular-acr-classification-criteria-in-juvenile-onset-systemic-lupus-erythematosus/