Preliminary diagnostic guidelines for macrophage activation syndrome complicating systemic juvenile idiopathic arthritis
Preliminary diagnostic guidelines for macrophage activation syndrome complicating systemic juvenile idiopathic arthritis
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DOI：
10.1016/j.jpeds.2004.12.016
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发表时间：
2005-05-01
影响因子：
5.1
通讯作者：
Martini, A
中科院分区：
文献类型：
--
作者：
Ravelli, A;Magni-Manzoni, S;Martini, A
目的探讨巨噬细胞活化综合征（MAS）合并全身性幼年特发性关节炎（S-JIA）的诊断标准。研究设计我们遵循的分类标准方法是基于对患有指示疾病的患者与患有“易混淆”疾病的患者进行比较。前一组包括74例文献报道或作者观察到的S-AA相关MAS患者;后一组包括37例S-JIA患者，作者观察到51例“高疾病活动性”。通过计算敏感率、特异率、受试者工作特征曲线下面积和诊断优势比（DOR），评价临床、实验室和组织病理学变量在区分MAS患者和高疾病活动性患者中的相对功效。通过“存在标准数”方法确定导致患者和对照受试者之间最佳分离的变量组合。（DOR = 67）和中枢神经系统功能障碍（DOR = 63）;最强的实验室鉴别因子是血小板计数降低（DOR = 1092）、天冬氨酸转氨酶升高（DOR = 247）、白细胞减少症（DOR = 70）和低纤维蛋白原血症（DOR = 165）。当任何2个或更多的实验室标准（DOR = 1309）同时存在时，患者和对照组之间的最佳分离发生;第二好的性能是由任何2，3，或更多的临床和/或实验室标准（DOR = 765和743，分别）的存在提供。这些指导方针值得前瞻性验证。
Objective To develop diagnostic guidelines for macrophage activation syndrome (MAS) complicating systemic juvenile idiopathic arthritis (S-JIA).Study design We followed the classification criteria approach that is based on the comparison of patients with the index disease with patients with a "confusable" disease. The former group included 74 patients with S-AA-associated MAS reported in the literature or seen by the authors; the latter group included 37 patients with S-JIA who had 51 instances of "high disease activity" seen by the authors. The relative power of clinical, laboratory, and histopathologic variables in discriminating patients with MAS from patients with high disease activity was evaluated by calculating the sensitivity rate, specificity rate, area under the receiver operating characteristic curve, and diagnostic odds ratio (DOR). The combinations of variables that led to best separation between patients and control subjects were identified through "the number of criteria present" method.Results The strongest clinical discriminators were hemorrhages (DOR = 67) and central nervous system dysfunction (DOR = 63); the strongest laboratory discriminators were decreased platelet count (DOR = 1092), increased aspartate aminotransferase (DOR = 247), leukopenia (DOR = 70), and hypofibrinogenemia (DOR = 165). The best separation between patients and control subjects occurred when any 2 or more laboratory criteria (DOR = 1309) were simultaneously present; the second best performance was provided by the presence of any 2, 3, or more clinical and/or laboratory criteria (DOR = 765 and 743, respectively).Conclusion We identified preliminary diagnostic guidelines for MAS complicating S-JIA. These guidelines deserve prospective validation.