Performance of the New 2019 European League Against Rheumatism/American College of Rheumatology Classification Criteria for Systemic Lupus Erythematosus in Children and Young Adults

Abstract

Objective. To compare the diagnostic usefulness of the 2019 European League Against Rheumatism (EULAR)/ American College of Rheumatology (ACR) classification criteria for systemic lupus erythematosus (SLE) to that of the 1997 ACR classification criteria for SLE when applied to youths (age ≤21 years) with SLE. Methods. Data were extracted from electronic health records of patients followed at a large academic pediatric hospital. The treating rheumatologist's diagnosis of SLE served as the standard criterion for identifying SLE patients (cases). Controls were patients with juvenile dermatomyositis (DM), juvenile scleroderma, or juvenile systemic sclerosis (SSc). The 2019 EULAR/ACR criteria and the 1997 ACR criteria were tested against the standard criterion. Results. A total of 112 SLE patients ages 2-21 years and 105 controls ages 1-19 years (66% juvenile DM, 34% juvenile scleroderma or juvenile SSc) were available for analysis. The 2019 EULAR/ACR criteria had significantly higher sensitivity (85% versus 72%; P = 0.023) and similar specificity (83% versus 87%; P = 0.456) than the 1997 ACR criteria. The mean ± SD 2019 EULAR/ACR classification summary scores were significantly higher among non-White than White patients (22.41 ± 10.04 versus 17.59 ± 9.19; P < 0.01). The sensitivity of the 2019 EULAR/ACR criteria in non-White/White patients was 92%/80% (P = 0.08) versus 83%/64% (P < 0.02) for the 1997 ACR criteria. The sensitivity of the 2019 EULAR/ACR criteria was not affected by age or sex. Conclusion. The 2019 EULAR/ACR criteria efficiently classify youths with SLE, irrespective of age, sex, and race. Compared to the 1997 ACR criteria, the new criteria are significantly more sensitive and similarly specific in youths with SLE.

Systemic Lupus Erythematosus in Children and Young People

“…The absolute EULAR/ACR-2019 classification summary scores were higher in non-White than White cases. Sub-analysis showed that the sensitivity of the EULAR/ ACR-2019 criteria was not influenced by patient ethnicity, age or gender, whereas the sensitivity of the ACR-1997 criteria was significantly higher in non-White versus White cases [147]. Further studies are warranted to assess the performance of the EULAR/ACR-2019 criteria in children, in particular as younger children with jSLE have been shown to display less ANA positivity [20].…”

An Update on the Management of Childhood-Onset Systemic Lupus Erythematosus

“…Three recent reports evaluated 2019-EULAR/ACR classification criteria in cSLE patients. The 2019-EULAR/ACR criteria effectively classify cSLE, irrespective of age, sex, and race, and these new criteria were more sensitive (85% vs 72%) with comparable specificity in youths with SLE compared with the 1997-ACR criteria (83% vs 87%) [ 91 ]. Another study showed the same sensitivity for 2019-EULAR/ACR and 2012-SLICC criteria (97.4%) and it was higher compared with the 1997-ACR criteria in cSLE patients (87.2%), with similar specificity between 2012-SLICC and 2019-EULAR/ACR criteria (98.4% and 99.7%, respectively) [ 92 ].…”

Limited sensitivity and specificity of the ACR/EULAR-2019 classification criteria for SLE in JSLE?—observations from the UK JSLE Cohort Study

“…Two recent studies have assessed the performance of ACR/EULAR-2019 criteria in JSLE [ 10 , 11 ]. The first study included 122 JSLE patients and 89 controls (ANA positive with other rheumatic diseases).…”

“…Data on performance of ACR/EULAR-2019 criteria in JSLE is limited to two relatively small cohorts that both suggested limited specificity when compared with ACR-1997 or SLICC-2012 criteria [ 10 , 11 ]. These studies did not include longitudinal assessment of the ACR/EULAR-2019 criteria.…”