The Performances of the ACR 1997, SLICC 2012, and EULAR/ACR 2019 Classification Criteria in Pediatric Systemic Lupus Erythematosus

Abstract

Objective Systemic lupus erythematosus (SLE) is a chronic autoimmune disease. The ACR (American College of Rheumatology) 1997, SLICC (Systemic Lupus International Collaborating Clinics) 2012, and EULAR (European League Against Rheumatism)/ACR 2019 SLE classification criteria are formed based on data mainly from adult patients. We aimed to test the performances of the SLE classification criteria among pediatric SLE patients. Methods Pediatric SLE patients (n=262; 80.9% female) were included from three different centers in Turkey. As controls, 174 children (60.9% female) with other diseases who had ANA (antinuclear antibody) test results were included. The gold standard for SLE diagnosis was expert opinion. Results The sensitivities of the ACR 1997, SLICC 2012, and EULAR/ACR 2019 criteria were 68.7%, 95.4%, and 91.6%, respectively. The specificities of the ACR 1997, SLICC 2012, and EULAR/ACR 2019 criteria were 94.8%, 89.7%, and 88.5%, respectively. 18 SLE patients met the SLICC 2012 but not the EULAR/ACR 2019 criteria. Among these, hematologic involvement was prominent (13/18; 72.2%). Eight SLE patients fulfilled the EULAR/ACR 2019 but not the SLICC 2012 criteria. Among these, joint involvement was prominent (6/8; 75%). Conclusion This is the largest cohort study of pediatric SLE testing the performances of all three classification criteria. The SLICC 2012 criteria yielded the best sensitivity, while the ACR 1997 criteria had the best specificity. SLICC 2012 criteria performed better than EULAR/ACR 2019 criteria. Separation of different hematological manifestations in the SLICC 2012 criteria might have contributed to the higher performance of this criteria set.

Evaluating the Performance of ACR, SLICC and EULAR/ACR Classification Criteria in Childhood onset Systemic Lupus Erythematosus

“…The result of our cohort correlates with a recent metaanalysis that highlights that these major organ involvement are more common in cSLE compared to adult onset SLE [20]. However, compared to other cSLE cohorts described in the literature, our cSLE cohort had a much younger age of disease onset of 7 (4-10) years compared to median age 10-13 years in other studies [13][14][15][16][17][18][19]. Perhaps, the lower age of transition to adult care in Arab health care due to cultural differences plays a role in having a younger cohort.…”

“…Our study highlights that there are various trends in performance of various SLE classi cation criteria among various studies [13][14][15][16][17][18]. Ethnicity and genetic ancestry play a major role in clinical presentation and outcome of SLE [19].…”

“…To date, there has been two other study populations in children comparing the performance of the three classi cation criteria [16,18]. Batu [14].…”

“…To date, studies comparing the performance of various classi cation criteria in cSLE are limited [13][14][15][16][17][18]. Most of the studies reported are from North America and Europe, however to date, none of the studies reported are of children of Arab ethnicity.…”

The expansion of activated naive DNA autoreactive B Cells and its association with disease activity in Systemic Lupus Erythematosus patients

“…Inclusion criteria for study subjects were: (1) a diagnosis of SLE based on Systemic Lupus International Collaborating Clinics (SLICC) criteria 2012 [24,25] Disease activity was measured by the modi ed SLEDAI-2K. Laboratory parameters (ESR, anti-dsDNA antibodies, C3 and C4 levels) were determined and correlated with aNAV B cells' frequency.…”