University of Cincinnati and Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.
Institute of Translational Medicine and Alder Hey Children's NHS Foundation Trust, Liverpool, UK.
Arthritis Care Res (Hoboken). 2018 Jun;70(6):813-822. doi: 10.1002/acr.23557. Epub 2018 Apr 25.
To validate the preliminary criteria of global flare for childhood-onset SLE (cSLE).
Pediatricians experienced in cSLE care (n = 268) rated unique patient profiles; results of standard cSLE laboratory testing and information about the cSLE flare descriptors were presented as follows: global assessment of patient well-being, physician global assessment of disease activity (MD-global), Disease Activity Index score, protein/creatinine ratio (PCR), and erythrocyte sedimentation rate (ESR). Using rater interpretation of the course of cSLE (baseline versus followup as the gold standard), performance (sensitivity, specificity, area under the receiver operating characteristic curve [AUC]) of the preliminary flare criteria was tested. An international consensus conference was held to rank the preliminary flare criteria as per the American College of Rheumatology recommendations and delineate threshold scores for minor, moderate, and major flares.
The accuracy of the 2 highest-ranked candidate criteria that consider absolute changes (∆) of the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) or British Isles Lupus Assessment Group (BILAG) (numeric scoring: A = 12, B = 8, C = 1, and D/E = 0), MD-global, PCR, and ESR were confirmed (both AUC >0.93). For the SLEDAI-based criteria (0.5 × ∆SLEDAI + 0.45 × ∆PCR + 0.5 × ∆MD-global + 0.02 × ∆ESR) flare scores ≥6.4/3.0/0.6 constituted major/moderate/minor flares, respectively. For the BILAG-based algorithm (0.4 × ∆BILAG + 0.65 × ∆PCR + 0.5 × ∆MD-global + 0.02 × ∆ESR) flare scores ≥7.4/3.7/2.2 delineated major/moderator/minor flares, respectively. These threshold values (SLEDAI, BILAG) were all >82% sensitive and specific for capturing flare severity.
Provisional criteria for global flares in cSLE are available to identify patients who experienced a flare. These criteria also allow for discrimination of the severity of cSLE exacerbations.
验证儿童发病系统性红斑狼疮（cSLE）全球疾病活动度的初步标准。
有儿童 SLE 诊治经验的儿科医生（n=268）对患者独特的病例资料进行了评分；标准的 cSLE 实验室检测结果和 cSLE 疾病活动度描述符的信息如下：患者整体健康状况评估、医生整体疾病活动度评估（MD-global）、疾病活动度指数评分、蛋白/肌酐比值（PCR）和红细胞沉降率（ESR）。根据 cSLE 病程的解读（以基线和随访为金标准），对初步疾病活动度标准的性能（灵敏度、特异性、受试者工作特征曲线下面积[AUC]）进行了检测。召开了一次国际共识会议，按照美国风湿病学会的建议对初步疾病活动度标准进行了排名，并划定了轻度、中度和重度疾病活动度的阈值评分。
2 个排名最高的候选标准的准确性得到了证实，这两个标准均考虑了系统性红斑狼疮疾病活动度指数（SLEDAI）或不列颠群岛狼疮评估组（BILAG）的绝对变化（∆）（数字评分：A=12、B=8、C=1 和 D/E=0）、MD-global、PCR 和 ESR（AUC 均>0.93）。基于 SLEDAI 的标准（0.5×∆SLEDAI+0.45×∆PCR+0.5×∆MD-global+0.02×∆ESR），疾病活动度评分≥6.4/3.0/0.6 分别为重度/中度/轻度疾病活动度。基于 BILAG 的算法（0.4×∆BILAG+0.65×∆PCR+0.5×∆MD-global+0.02×∆ESR），疾病活动度评分≥7.4/3.7/2.2 分别为重度/中度/轻度疾病活动度。这些阈值（SLEDAI、BILAG）对于识别疾病活动度恶化的患者均有>82%的灵敏度和特异性。
儿童发病系统性红斑狼疮的全球疾病活动度的初步标准已经确定，这些标准可用于识别发生疾病活动度恶化的患者，也可用于区分儿童发病系统性红斑狼疮加重的严重程度。
