Yokoyama Tadafumi, Shimizu Masaki, Ikeno Iku, Hashida Yoko, Fujita Syuhei, Shimura Shoetu, Hatasaki Kiyoshi, Shintani Naohisa, Yachie Akihiro
Department of Pediatrics, School of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University.
Nihon Rinsho Meneki Gakkai Kaishi. 2011;34(2):105-11. doi: 10.2177/jsci.34.105.
The differential diagnosis of macrophage activation syndrome (MAS) and sepsis must be considered in the clinical course of systemic-onset juvenile idiopathic arthritis (s-JIA) with sudden onset of high-grade fever and abnormal laboratory findings, including leukocytopenia, thrombocytopenia, and coagulopathy. In this report, we describe the case of a 17-month-old girl diagnosed with s-JIA complicated with sepsis. Her serum interleukin (IL)-18 level was significantly elevated throughout the clinical course. Furthermore, compared to other MAS patients, she showed a significantly elevated serum IL-6 level and procalcitonin in sepsis. Therefore, our results suggest that a patient's cytokine profile may be a useful indicator of disease activity and may thus help in the differential diagnosis of sepsis and MAS in s-JIA.
在全身型幼年特发性关节炎（s-JIA）临床病程中，若突然出现高热及实验室检查异常（包括白细胞减少、血小板减少和凝血功能障碍），必须考虑巨噬细胞活化综合征（MAS）与脓毒症的鉴别诊断。在本报告中，我们描述了一名17个月大诊断为s-JIA并发脓毒症的女孩病例。在整个临床病程中，她的血清白细胞介素（IL）-18水平显著升高。此外，与其他MAS患者相比，她在脓毒症时血清IL-6水平和降钙素原显著升高。因此，我们的结果表明，患者的细胞因子谱可能是疾病活动的有用指标，从而有助于s-JIA中脓毒症和MAS的鉴别诊断。
