There is no significant difference in sensitivity and specificity between the 2017 weighted criteria and the 2012 Systemic Lupus International Collaborating Clinics (SLICC) criteria among patients with pediatric systemic lupus erythematosus (SLE), according to research results published in Arthritis Care and Research.

Researchers conducted a retrospective chart review of patients with pediatric SLE to validate and compare the sensitivity and specificity of the 2017 weighted criteria with the 1997 American College of Rheumatology (ACR) and 2012 SLICC criteria.

The review included patients (82.7% girls) between 2003 and 2018 who were diagnosed before their 19th birthday (mean age at diagnosis, 13.1±2.8 years); mean disease duration at follow-up was 49.7±36.3 months. Researchers analyzed the charts of 156 patients with pediatric SLE and compared them with the charts of 379 patients with other autoimmune diseases; patients in the control group included those with positive antinuclear antibodies but no diagnosis of SLE, or with another autoimmune disease (juvenile idiopathic arthritis, linear scleroderma, Sjogren syndrome, or juvenile dermatomyositis).

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Researchers applied 3 criteria sets to data available in all charts. More than 87% of patients (n=136) with SLE were classified as SLE-positive according to the 2017 weighted, 1997 ACR, and 2012 SLICC criteria; 9.6% (n=15) were classified as SLE-positive according to the 2017 weighted and 2012 SLICC criteria only, 1.9% (n=3) were not classified as SLE-positive according to any criteria, and 2 remaining patients were classified according to either the 2017 weighted or the 2012 SLICC criteria only. In comparison, among patients in the autoimmune control group, 98.4% were classified as SLE-negative according to all 3 criteria, 1.3% were classified as SLE-positive according to the 2017 weighted criteria only, and 0.3% were classified as SLE-positive according to both the 2017 weighted and the 2012 SLICC criteria.

Patients classified according to the 2017 weighted criteria fulfilled a median of 6 of 11 domains (range, 2-9 domains) and scored a median of 21 points (range, 6-41 points). Sensitivity was 0.974 (95% exact CI, 0.936-0.993), and specificity was 0.984 (95% exact CI, 0.966-0.994). The most common domains were antibodies and complement, satisfied by 97.4% (n=152) and 82.1% (n=128) of SLE-positive patients, respectively; 64.1% (n=100) and 60.3% (n=94) of patients met hematologic and synovitis clinical criteria, respectively.

Patients classified according to the 1997 ACR criteria satisfied a median of 4 of 11 criteria (range, 2-7). Sensitivity was 0.872 (95% exact CI, 0.809-0.920), and specificity was 1.0 (95% exact CI, 0.990-1.000, respectively). In SLE-positive patients, 97.4% (n=152) had immunologic manifestations, 66.7% (n=104) had hematologic disease, and 60.3% (n=94) had arthritis. Additional criteria included malar rash, renal disease, oral ulcers, and serositis. In the SLE-negative group, 54% (n=204) of patients had arthritis, and the remaining criteria were met by less than 5% of patients.

Patients classified according to the 2012 SLICC criteria satisfied a median of 7 of 17 criteria (range, 2-12). Sensitivity was 0.974 (95% exact CI, 0.936-0.993), and specificity was 0.997 (95% exact CI, 0.985-1.000, respectively). In SLE-positive patients, the 3 most commonly met immunologic criteria were anti-dsDNA, complement, and anti-Smith antibodies (met by 89.1% [n=139], 82.1% [n=128], and 68.6% [n=107], respectively). Synovitis was the most frequently met clinical criteria (60.3%; n=94). Of the 379 SLE-negative patients, 164 (43%) had synovitis, and the remaining were met by less than 5% of patients

“Our study shows that the 2017 weighted and 2012 SLICC are more sensitive, but similarly specific to the 1997 ACR criteria,” the researchers concluded. “We plan to further evaluate the performances of these criteria in a larger cohort of patients.”

Reference

Ma M, Hui-Yuen JS, Cerise JE, Iqbal S, Eberhard BA. Validation of the 2017 weighted criteria compared to the 1997 ACR and the 2012 SLICC in pediatric systemic lupus erythematosus [published online September 3, 2019]. Arthritis Care Res. doi:10.1002/acr.24057