Researchers validated the 2019 American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) classification criteria for diagnosing systemic lupus erythematosus (SLE) in children and youth, and it was found to be more sensitive and similarly specific compared to the 1997 ACR classification criteria, according to a brief report published in Arthritis Care & Research.

In 2019, the EULAR and ACR developed new classification criteria for SLE to replace the 1997 ACR criteria. The new criteria — developed to improve the diagnosis of SLE —  include the diverse spectrum of SLE manifestations, use a weighted scoring system, and require an antinuclear antibody titer of at least 1:80 on human epithelial type 2 (HEp-2) cells or an equivalent positive test.

The current study assesses the performance of the 2019 EULAR/ACR criteria in children and young adults aged 21 years or younger.


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Using data extracted from the electronic medical records from a large pediatric hospital, the researchers identified patients aged 21 years or younger with a diagnosis of SLE using the International Classification of Diseases, Tenth Revision (ICD-10) codes. Patients with diagnoses of juvenile dermatomyositis, juvenile scleroderma, and juvenile systemic sclerosis were also identified as control participants in the current analysis, as they demonstrated similar clinical and laboratory features with SLE. Clinical data were used to score patients using the 2019 EULAR/ACR and 1997 ACR criteria. Sensitivity and specificity analyses were conducted to assess the 2019 and 1997 criteria against the standard criterion (ICD-10 diagnosis).

The study included 217 participants (112 with SLE and 105 control participants with a diagnosis of juvenile dermatomyositis, juvenile scleroderma, or juvenile systemic sclerosis). The sensitivity of the 2019 EULAR/ACR criteria was found to be significantly higher than the 1997 ACR criteria (85% vs 72%; P =.023). There were no significant differences in specificity (P =.456). The sensitivity was higher among non-White vs White patients, using both the 2019 EULAR/ACR criteria (92% vs 80%; P =.08) and the 1997 ACR criteria (83% vs 64%; P <.02). This difference was likely due to the more severe disease presentation at diagnosis in non-White patients, as evidenced by the higher 2019 EULAR/ACR classification summary scores in non-White vs White patients (22.41±10.04 vs 17.59±9.19, respectively; P <.01). The sensitivity of the 2019 EULAR/ACR criteria was not affected by age or sex.

One limitations of the study was that the lack of inclusion of other SLE criteria sets, such as the 2012 Systemic Lupus International Collaborating Clinics (SLICC) classification criteria.

“The 2019 EULAR/ACR criteria efficiently classify youths with SLE, irrespective of age, sex, and race,” the researchers noted. “Our study adds insight about the performance of the new 2019 EULAR/ACR criteria in the clinical setting for youths with SLE and emphasizes the importance of these criteria in classification of patients for research.”

Reference

Aljaberi N, Nguyen K, Strahle C, Merritt A, Mathur A, Brunner HI. Performance of the new 2019 European League Against Rheumatism/American College of Rheumatology Classification Criteria for systemic lupus erythematosus in children and young adults. Arthritis Care Res. Published online March 26, 2021. doi:10.1002/acr.24430