POS0136 PERFORMANCE OF THE AMERICAN COLLEGE OF RHEUMATOLOGY-1997, THE SYSTEMIC LUPUS INTERNATIONAL COLLABORATING CLINICS-2012, AND THE EUROPEAN LEAGUE AGAINST RHEUMATISM-2019 CRITERIA FOR CHILDHOOD-ONSET SYSTEMIC LUPUS ERYTHEMATOSUS: A SOUTHEAST ASIA VALIDATION STUDY

Background

Classification criteria for systemic lupus erythematosus (SLE) include the American College of Rheumatology (ACR) 1997, the Systemic Lupus International Collaborating Clinics (SLICC) and the European League Against Rheumatism (EULAR)/ACR criteria^[1]. Their performance in a primarily Asian childhood-onset SLE (cSLE) population remains unclear as the clinical manifestation frequencies are different^[2].

Objectives

To evaluate the diagnostic performance of ACR-1997, SLICC-2012, and EULAR/ACR-2019 criteria in a cSLE cohort in Singapore.

Methods

Cases were physician-diagnosed cSLE, while controls were children with mixed and undifferentiated connective tissue disease that posed an initial diagnostic challenge. Retrospective data were reviewed to establish the three criteria fulfilled at diagnosis and over time.

Results

The study population included 120 cSLE cases and 36 controls, Table 1. Among cSLE patients, low C3/4 (94.2%), immunologic (93.3%), hematologic manifestations (90.0%) and arthritis (60.8%) were the most common over time. At diagnosis, 102 (85%) patients fulfilled all criteria, Figure 1. SLICC-2012 had the highest sensitivity (97.5%) and negative predictive value (NPV, 89.3%), while ACR-1997 had the highest specificity (91.7%) and positive predictive value (PPV, 97.2%). All criteria had diagnostic accuracies of ≥ 85%. Over time, 113 (94.2%) fulfilled all criteria. SLICC-2012 remained the criteria with highest sensitivity (99.2%) and NPV (94.7%), while ACR-1997 remained the criteria with highest specificity (75.0%) and PPV (92.7%). Only SLICC-2012 and ACR-1997 had ≥ 85% diagnostic accuracy over time.

Conclusion

This is the first study evaluating the SLE classification criteria performance in Southeast Asian cSLE cohort. SLICC-2012 criterion has the highest sensitivity and diagnostic accuracy, with acceptable specificity in our cohort, especially early in the disease course. The EULAR/ACR-2019 criteria does not confer additional benefits compared to the earlier two criteria.

References

[1]Chang, L. S., Huang, P. Y., Kuo, H. C., Tu, Y. K., Tseng, P. T., Liang, C. S., & Hsu, C. W. (2022). 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. Autoimmunity Reviews, 103144. [2]Tang, S. P., Lim, S. C., & Arkachaisri, T. (2021). Childhood-onset systemic lupus erythematosus: Southeast Asian perspectives. Journal of Clinical Medicine, 10(4), 559.

Acknowledgements:

NIL.

Disclosure of Interests

None Declared.