Comparison among ACR1997, SLICC and the new EULAR/ACR classification criteria in childhood-onset systemic lupus erythematosus

Abstract

Background: To date there are no specific classification criteria for childhood-onset systemic lupus erythematosus (cSLE). This study aims to compare the performance among the American College of Rheumatology (ACR) 1997, the Systemic Lupus International Collaborating Clinics criteria (SLICC) and the new European League Against Rheumatism (EULAR)/ACR criteria, in a cSLE cohort. Methods: We conducted a medical chart review study of cSLE cases and controls with defined rheumatic diseases, both ANA positive, to establish each ACR1997, SLICC and EULAR/ACR criterion fulfilled, at first visit and 1-yearfollow-up. Results: Study population included 122 cSLE cases and 89 controls. At first visit, SLICC criteria had higher sensitivity than ACR 1997 (89.3% versus 70.5%, p < 0.001), but similar specificity (80.9% versus 83.2%, p = 0.791), however performance was not statistically different at 1-year-follow-up. SLICC better scored in specificity compared to EULAR/ACR score ≥ 10 at first visit (80.9% versus 67.4%, p = 0.008) and at 1-year (76.4% versus 58.4%, p = 0.001), although sensitivities were similar. EULAR/ACR criteria score ≥ 10 exhibited higher sensitivity than ACR 1997 (87.7% versus 70.5%, p < 0.001) at first visit, but comparable at 1-year, whereas specificity was lower at first visit (67.4% versus 83.2%, p = 0.004) and 1-year (58.4% versus 76.4%, p = 0.002). A EULAR/ACR score ≥ 13 against a score ≥ 10, resulted in higher specificity, positive predictive value, and cutoff point accuracy. Compared to SLICC, a EULAR/ACR score ≥ 13 resulted in lower sensitivity at first visit (76.2% versus 89.3%, p < 0.001) and 1-year (91% versus 97.5%, p = 0.008), but similar specificities at both assessments. When compared to ACR 1997, a EULAR/ACR total score ≥ 13, resulted in no differences in sensitivity and specificity at both observation periods. Conclusions: In this cSLE population, SLICC criteria better scored at first visit and 1-year-follow-up. The adoption of a EULAR/ACR total score ≥ 13 in this study, against the initially proposed ≥10 score, was most appropriate to classify cSLE. Further studies are necessary to address if SLICC criteria might allow fulfillment of cSLE classification earlier in disease course and may be more inclusive of cSLE subjects for clinical studies.

Reply

“…We are grateful to Fonseca and colleagues for their efforts to test the new criteria in a South American pediatric population and for their reiteration that leukopenia in patients of African descent is common and may lead to misclassification if not correctly attributed to ethnicity. The significant prevalence of SLE‐specific items in their non‐SLE patient population , however, sheds doubt on the correct use of the EULAR/ACR 2019 SLE classification criteria attribution rule or, less likely, the non‐SLE diagnoses in this cohort. This may explain some of the discrepancies between their cohort and the EULAR/ACR SLE criteria validation cohort.…”

“…While unfortunately details on the exact distribution of the EULAR/ACR criteria items were not provided by Fonseca et al , the data on the SLICC criteria items listed suggest that item attribution for the EULAR/ACR criteria may not have been correctly performed. For example, 25% of the non‐SLE patients in their sample had an acute cutaneous lupus erythematosus (ACLE) rash . If this were true ACLE by the EULAR/ACR definition (“Malar rash or generalized maculopapular rash observed by a clinician.…”

“…Dr. Fonseca and colleagues refer to their analysis of the new EULAR/ACR 2019 criteria in a group of 122 patients with childhood‐onset SLE and 89 patients with other diagnoses mimicking SLE . This analysis was published before the full contents of the EULAR/ACR 2019 criteria were available, which may explain why some important aspects were not taken into account.…”

Does Leukopenia Influence Performance of the New European League Against Rheumatism/American College of Rheumatology Classification Criteria in an African‐Descendent Population With Childhood‐Onset Systemic Lupus Erythematosus? Comment on the Article by Aringer et al

“…Our group recently published a study that assessed the performance of the new EULAR/ACR classification criteria in a population with childhood‐onset SLE and controls with other defined rheumatic diseases that often are difficult to distinguish from childhood‐onset SLE in daily clinical practice . Both our childhood‐onset SLE group and the control patients were all positive for ANA, which may constitute an external validation since the original derivation and validation cohorts did not include patients with childhood‐onset SLE, as stated by Aringer et al .…”

A comparison and review of three sets of classification criteria for systemic lupus erythematosus for distinguishing systemic lupus erythematosus from pure mucocutaneous manifestations in the lupus disease spectrum

“…A recent study reported that, if the cut-off score was changed to 13, it would result in a higher specificity, positive predictive value, and cut-off point accuracy. 15 Therefore, in this study, we also desired to explore proper modification of EULAR/ACR’19.…”