Evaluating the performance of ACR, SLICC and EULAR/ACR classification criteria in childhood onset systemic lupus erythematosus

Abstract

Background The ACR 1997, SLICC 2012 and EULAR/ACR 2019 classification criteria were validated based on adult patients. To date, there are no classification criteria specific for children with SLE. The aim of the study is to compare the performance characteristics among the three SLE classification criteria (ACR-1997, SLICC-2012 and EULAR/ACR-2019) in childhood onset SLE (cSLE) cohort of Arab ethnicity from Oman. Methods We conducted a retrospective multicenter study in Oman of cSLE patients as cases and patients with other rheumatic disease with a positive ANA titer as controls. The cSLE cases recruited were children diagnosed with SLE before 13 years of age. Data was retrospectively collected to establish the ACR-1997, SLICC-2012 and EULAR/ACR-2019 criteria fulfilled at first visit, first year follow up and last follow up. Results Study population included 113 cSLE cases (mean age at diagnosis of 7.3 ± 3.4 years with disease duration of 6.1 ± 4.6 years) and 51 controls (mean age at diagnosis 5.0 ± 3.4 with disease duration 5.7 ± 3.9). The cSLE cases had higher frequency of familial SLE than controls (38% vs 7.8%; p < 0.001). The performance measures demonstrated that EULAR/ACR-2019 criteria had the highest sensitivity (81, 88, 89%) compared to ACR 1997 (49, 57, 66%) and SLICC 2012 (76, 84,86%); while the ACR 1997 had the highest specificity (96%) compared to SLICC 2012 (94%) and EULAR/ACR 2019 (90%) at first visit, first year and last assessment. When we increased the threshold score to ≥13 rather than the traditional score ≥ 10 for ACR/EULAR 2019, there was increased specificity (96%) at the expense of lower sensitivity (76, 83, and 84%) at first visit, first year and last assessment. Conclusion In this cSLE population, EULAR/ACR 2019 scored better at initial presentation, first year and last assessment follow up. Further multinational studies are needed to validate the appropriate cut off score for the newly proposed ACR/EULAR 2019 classification criteria in cSLE to increase early sensitivity and specificity for cSLE classification.

Evaluating performance of the 2019 EULAR/ACR, 2012 SLICC, and 1997 ACR criteria for classifying adult-onset and childhood-onset systemic lupus erythematosus: A systematic review and meta-analysis

“…After deleting duplicates, 29 studies met the inclusion criteria ( Figure 1 ). Of these, 18 studies ( 17 , 18 , 25 , 26 , 35 – 48 ) included patients with adult-onset SLE, and 11 studies ( 21 , 22 , 27 , 29 , 30 , 49 – 54 ) included patients with childhood-onset SLE. For adult-onset SLE, 10 studies ( 17 , 25 , 26 , 38 , 41 , 42 , 45 – 48 ) assessed the three criteria (ACR 1997, SLICC 2012, and EULAR/ACR 2019), six studies ( 18 , 36 , 37 , 39 , 43 , 44 ) assessed two criteria (ACR 1997 and SLICC 2012), and two studies ( 35 , 40 ) assessed only one set of criteria (ACR 1997).…”

“…For adult-onset SLE, 10 studies ( 17 , 25 , 26 , 38 , 41 , 42 , 45 – 48 ) assessed the three criteria (ACR 1997, SLICC 2012, and EULAR/ACR 2019), six studies ( 18 , 36 , 37 , 39 , 43 , 44 ) assessed two criteria (ACR 1997 and SLICC 2012), and two studies ( 35 , 40 ) assessed only one set of criteria (ACR 1997). Six childhood-onset SLE studies ( 27 , 29 , 30 , 52 – 54 ) evaluated all three criteria (ACR 1997, SLICC 2012, and EULAR/ACR 2019), and five studies ( 21 , 22 , 49 – 51 ) evaluated two criteria (four studies evaluated ACR 1997 and SLICC 2012, and one study evaluated ACR 1997 and EULAR/ACR 2019). Only two included studies had more than one reference standard (one adult-onset and one childhood-onset SLE study).…”

“…Some authors have found that EULAR/ACR 2019 is not the best tool for childhood-onset SLE diagnosis compared with SLICC 2012 and ACR 1997 because it has lower sensitivity than SLICC 2012 and lower specificity than ACR 1997 ( 27 , 28 ). However, some studies have found that EULAR/ACR 2019 performs better and improves over time compared with SLICC 2012 and ACR 1997 ( 29 , 30 ).…”

An Overview of Systemic Lupus Erythematosus (SLE) Pathogenesis, Classification, and Management

“…To rectify this, the 2012 SLICC was introduced with improved sensitivity of 97% and an increase in sensitivity to 84% early in the disease. However, the specificity decreased to 84%, whereas ACR criteria specificity was 93% [ 58 ].…”

Correction to: Evaluating the performance of ACR, SLICC and EULAR/ACR classification criteria in childhood onset systemic lupus erythematosus

“…Following publication of the original article [ 1 ], the author identified an error in the author name of Eman Al Masroori. The incorrect author name is: Eiman Masroori The correct author name is: Eman Al Masroori …”