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Limited sensitivity and specificity of the ACR/EULAR-2019 classification criteria for SLE in JSLE?-observations from
Eve M D Smith1,2, Sajida Rasul3, Coziana Ciurtin4
1Department of Women's & Children's Health, Institute of Life Course and Medical Sciences, University of Liverpool.
Insights
The new ACR/EULAR-2019 criteria for juvenile idiopathic arthritis (JIA) show no superiority over existing SLICC-2012 or ACR-1997 criteria in children and young people (CYP). Paediatric rheumatologists
Area of Science:
- Rheumatology
- Pediatric Rheumatology
- Immunology
Background:
- Juvenile idiopathic arthritis (JIA) classification criteria are crucial for diagnosis and research.
- The American College of Rheumatology (ACR) and European League Against Rheumatism (EULAR) updated criteria in 2019, incorporating antinuclear antibody (ANA) positivity.
- Evaluating these new criteria in pediatric populations is essential.
Purpose of the Study:
- To assess the performance of the new ACR/EULAR-2019 classification criteria in children and young people (CYP) with JIA.
- To compare the ACR/EULAR-2019 criteria against established SLICC-2012 and ACR-1997 criteria.
- To determine the sensitivity and specificity of these criteria in a pediatric cohort.
Main Methods:
- A retrospective analysis of the UK JIA Cohort Study data was conducted, including 482 CYP.
- The performance of ACR/EULAR-2019 criteria was compared with SLICC-2012 criteria, using ACR-1997 as the reference standard.
- Positive and negative predictive values were calculated using an unselected cohort of 129 ANA-positive CYP.
Main Results:
- Both ACR/EULAR-2019 and SLICC-2012 criteria showed significant differences in patient classification at first and last visits.
- SLICC-2012 criteria demonstrated higher sensitivity (98%) compared to ACR/EULAR-2019 (94-96%) across visits.
- ACR/EULAR-2019 criteria exhibited greater specificity (77-81%) than SLICC-2012 (67-71%), with highest false-positive rates in ANA-positive CYP (5%).
Conclusions:
- The ACR/EULAR-2019 criteria are not superior to SLICC-2012 or ACR-1997 criteria in classifying pediatric JIA.
- Differences in classification performance are influenced by ANA positivity variations across age groups.
- Inclusion of pediatric rheumatologists in the consensus and evaluation of classification criteria is vital for accurate outcomes in CYP.
Objectives:
This study aimed to test the performance of the new ACR and EULAR criteria, that include ANA positivity as entry criterion, in 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 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%).
Conclusion:
In 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.
