Yang Ji Won, Lee Eunyoung, Seo Ji-Yeon, Jung Ju-Yang, Suh Chang-Hee, Kim Hyoun-Ah
Department of Rheumatology, Ajou University School of Medicine, 164 Worldcup-ro, Yeongtong-gu, Suwon, 16499, South Korea.
Department of Biomedical Informatics, Ajou University School of Medicine, 164 Worldcup-ro, Yeongtong-gu, Suwon, 16499, South Korea.
Pediatr Rheumatol Online J. 2018 Jan 25;16(1):9. doi: 10.1186/s12969-018-0225-1.
Adult-onset Still's disease (AOSD) is an adult form of systemic juvenile idiopathic arthritis (JIA) that differs from the latter in its classification. This study evaluated the concordance between the International League Against Rheumatism (ILAR) criteria for systemic JIA and the Yamaguchi criteria and then compared their possible prognostic value in patients with AOSD.
In a retrospective review of 169 adults with suspected AOSD, patients were classified according to the Yamaguchi or ILAR criteria. Then the concordance in cross-referencing the other group with the different criteria was investigated and the sensitivity and specificity of each set of criteria were determined. Disease activity markers in AOSD patients were correlated with positivity according to both systems.
Concordance was good in patients with suspected AOSD (k = 0.7144, p < 0.001) and low in those with a diagnosis of AOSD (k = 0.3787, p < 0.001). The sensitivity of the ILAR criteria in AOSD patients was 0.8864 (95% confidence interval (CI): 0.8322-0.9405), and the specificity was 0.7838 (0.6511, 0.9164). Positivity according to the ILAR criteria correlated with the systemic score (r = 0.763, p < 0.0001) and C-reactive protein levels (r = 0.183, p = 0.0356) and was associated with a relapse (odds ratio: 1.589, 95% CI: 1.043-2.421), macrophage activation syndrome (MAS; odds ratio: 1.993, 95% CI: 1.218-3.263) and care in the intensive care unit (ICU; odds ratio: 2.087, 95% CI: 1.086-4.011).
In AOSD patients, there is fair concordance between the Yamaguchi and ILAR criteria for systemic JIA. Positive ILAR criteria may be useful for identifying AOSD patients at high risk for relapse, MAS and the need for ICU care. Further studies including larger populations from several centers are needed to confirm our results regarding the utility of the ILAR criteria in AOSD patients.
成人斯蒂尔病（AOSD）是系统性幼年特发性关节炎（JIA）的成人形式，在分类上与后者不同。本研究评估了系统性JIA的国际抗风湿病联盟（ILAR）标准与山口标准之间的一致性，然后比较了它们在AOSD患者中的可能预后价值。
在对169例疑似AOSD的成人患者进行回顾性研究时，根据山口标准或ILAR标准对患者进行分类。然后调查将另一组与不同标准交叉对照时的一致性，并确定每组标准的敏感性和特异性。根据这两种标准，将AOSD患者的疾病活动标志物与阳性结果进行关联分析。
疑似AOSD患者的一致性良好（k = 0.7144，p < 0.001），确诊为AOSD的患者一致性较低（k = 0.3787，p < 0.001）。ILAR标准在AOSD患者中的敏感性为0.8864（95%置信区间（CI）：0.8322 - 0.9405），特异性为0.7838（0.6511，0.91６4）。根据ILAR标准的阳性结果与全身评分（r = 0.763，p < 0.0001）和C反应蛋白水平（r = 0.183，p = 0.0356）相关，并与复发（优势比：1.589，95%CI：1.043 - 2.421）、巨噬细胞活化综合征（MAS；优势比：1.993，95%CI：1.218 - 3.263）以及在重症监护病房（ICU）接受治疗（优势比：2.087，95%CI：1.086 - 4.011）相关。
在AOSD患者中，系统性JIA的山口标准与ILAR标准之间有较好的一致性。ILAR标准阳性可能有助于识别有复发、MAS风险及需要ICU治疗的AOSD高危患者。需要进一步开展包括来自多个中心的更大规模人群的研究，以证实我们关于ILAR标准在AOSD患者中效用的研究结果。
