Clinical value of 2019 European League Against Rheumatism/American College of Rheumatology classification criteria in childhood-onset systemic lupus erythematosus / 中华实用儿科临床杂志

ABSTRACT

Objective:To compare the performance among the American College of Rheumatology (ACR) 1997, the Systemic Lupus International Collaborating Clinics (SLICC) 2012 and the 2019 European League Against Rheumatism (EULAR)/ACR criteria, in a childhood-onset systemic lupus erythematosus(CSLE) cohort.Methods:A medical chart review study was conducted of 182 cases of SLE patients and 163 controls with defined rheumatic diseases in pediatrics department of Renji Hospital, Shanghai Jiaotong University School Medicine, from January 2013 to May 2017, to establish each ACR1997, SLICC2012 and 2019EULAR/ACR criterion.The performance of the three criteria was statistically analyzed.Results:(1) Comparing the patients with SLE and controls, the difference in fever(21.4% vs.8.0%), skin lesions(54.9% vs.31.9%), nonscarring alopecia(3.8% vs.0), renal disorder(41.2% vs.5.5%), neurologic disorder(7.7% vs.1.8%), hematologic disorder [leukopenia(32.4% vs.1.8%), thrombocytopenia(31.9% vs.0)], low complement(83.5% vs.12.9%), anti-nuclear antibody(98.4% vs.23.3%), anti-dsDNA antibody(94.0% vs.8.6%), anti-Sm antibody(19.2% vs.0%), and antiphospholipid antibodies(16.5% vs.3.7%)had statistical significance (all P<0.05). But the difference in oral ulcers, synovitis, serositis and positive Coombs test had no statistical significance (all P>0.05). (2) Sensitivities of ACR1997, SLICC2012 and 2019EULAR/ACR criteria were 67.0%(122/182 cases), 95.6% (174/182 cases)and 97.8% (178/182 cases)( P<0.001), with specificities 99.4%(162/163 cases), 98.2% (160/163 cases)and 94.5%(154/163 cases) ( P=0.016), respectively.In terms of accuracy, the three classifications were 82.3%(284/345 cases), 96.8% (334/345 cases)and 96.2%(332/345 cases), respectively, the difference was statistically significant ( P<0.001). (3) Only 120 cases (65.9%) of patients with SLE met all 3 criteria.Eight cases of SLE patients who only met the 2019EULAR/ACR criteria exhibit high rate of single organ involvement (7 cases). Four cases of SLE patients were missed by the 2019EULAR/ACR, 3 cases of which were antinuclear antibody negative.(4) The SLICC2012 and 2019EULAR/ACR criteria had increased sensitivity for major organ damage than ACR1997.The total score of 2019 EULAR/ACR criteria correlated positively with SLE disease activity ( R2= 0.451, P<0.001). Conclusions:In this SLE population, the 2019EULAR/ACR criteria is more sensitive than ACR1997 and SLICC2012 criteria, allowing earlier classification and recognition of patients with single or major organ damage.Although the specificity is slightly lower than the previous two criteria, it is still worthy of clinical promotion.