摘要： 目的 探讨全身型幼年特发性关节炎（sJIA）患儿发生巨噬细胞活化综合征（MAS）的早期预警指标。方法 选取2015年1月至2021年9月深圳市儿童医院风湿免疫科住院的14例sJIA合并MAS患儿作为sJIA-MAS组。选取同期本科室临床资料相对齐全、初次诊断、未使用激素治疗的36例单纯sJIA患者作为对照（sJIA组）。比较2组患者各项临床指标的差异，并通过受试者操作特征（ROC）曲线分析，找出预测sJIA合并MAS各指标的截断值。结果 与sJIA组比较，sJIA-MAS组肝脾大，转氨酶升高，中枢神经系统症状，骨髓嗜血现象，儿童重症监护病房入住概率均显著增大（均P<0.05）；白细胞计数、中性粒细胞计数、淋巴细胞计数、血小板计数、白蛋白、纤维蛋白原显著降低（均P<0.05）；降钙素原、血清铁蛋白（SF）、谷丙转氨酶、谷草转氨酶、乳酸脱氢酶（LDH）、甘油三酯、铁蛋白/红细胞沉降率（SF/ESR）、乳酸脱氢酶/红细胞沉降率（LDH/ESR）、γ干扰素（IFN-γ）、白细胞介素-10（IL-10）显著升高（均P<0.05）。ROC曲线分析结果显示，sJIA-MAS患儿SF/ESR、LDH、LDH/ESR、SF的曲线下面积（AUC）分别为0.992、0.988、0.974、0.964；IFN-γ、IL-10的AUC分别为0.891、0.773。结论 当sJIA患者出现肝和（或）脾大、转氨酶升高、中枢神经系统症状和骨髓嗜血现象时，需考虑合并MAS。sJIA患儿在IL-6升高时出现IL-10、IFN-γ升高是发生MAS的预警信号。当sJIA患儿SF/ESR≥65.23、LDH≥448.50U/L、LDH/ESR≥5.22、SF≥6900.00ng/mL、IFN-γ≥12.32pg/mL、IL-10≥10.13pg/mL时应警惕MAS发生。

全身型幼年特发性关节炎患儿发生巨噬细胞活化综合征的早期预警指标

石晓萌, 罗颖, 何庭艳, 夏宇, 杨军

中国医科大学深圳市儿童医院风湿免疫科, 广东深圳 518038

收稿日期:2022-06-07出版日期:2023-06-30发布日期:2023-05-31
通讯作者:杨军E-mail:rogasansz@163.com
作者简介:石晓萌（1997-），女，医师，硕士研究生



关键词: 全身型幼年特发性关节炎, 巨噬细胞活化综合征, 早期预警指标
Abstract: Objective To investigate the cytokine profile of systemic juvenile idiopathic arthritis (sJIA)-associated macrophage activation syndrome (MAS),and assess the early warning laboratory indicators of MAS in sJIA. Methods The clinical data of 14 patients with sJIA complicated with MAS (sJIA-MAS group) admitted to the Department of Rheumatology and Immunology of Shenzhen Children's Hospital from January 2015 to September 2021 were collected and compared with 36 patients with sJIA without glucocorticoid treatment (sJIA group) admitted during the same period. Differences in clinical characteristics between both groups were compared via the difference analysis. Receiver operating characteristic analysis was used to predict the cut-off values of the laboratory indicators of MAS in sJIA. Results The incidence of hepatosplenomegaly,high levels of serum transaminases,central nervous system symptoms,bone marrow hematophagy,and pediatric intensive care unit occupancy rate was higher in the sJIA-MAS group compared with that in the sJIA group (all P < 0.05). White blood cell,neutrophil,lymphocyte,and platelet counts and albumin and fibrinogen levels were decreased,while procalcitonin,serum ferritin (SF),alanine aminotransferase,aspartate aminotransferase,lactate dehydrogenase,triglyceride,SF/erythrocyte sedimentation rate (ESR),lactate dehydrogenase (LDH)/ESR,interferon-γ (IFN-γ),and interleukin (IL)-10 levels were significantly higher in the sJIA-MAS group (all P < 0.05). The area under curve (AUC) of SF/ESR,LDH,LDH/ESR,and SF in patients with sJIA-MAS was 0.992,0.988,0.974,and 0.964,respectively. The AUC of IFN-γ and IL-10 in patients with sJIA-MAS was 0.891 and 0.773,respectively. Conclusion Presence of hepatosplenomegaly,high levels of the serum transaminases,abnormal central nervous system symptoms,and bone marrow hematophagy in patients with sJIA should raise the suspicion of MAS. Further,elevated levels of IL-10 and IFN-γ are early warning signals of MAS in children with sJIA based on elevated IL-6 levels. SF/ESR ≥ 65.23,LDH ≥ 448.50 U/L,LDH/ESR ≥ 5.22, SF ≥ 6 900.00 ng/mL,IFN-γ ≥ 12.32 pg/mL,and IL-10 ≥ 10.13 pg/mL can be used to predict the occurrence of MAS in sJIA.
Key words: systemic juvenile idiopathic arthritis, macrophage activation syndrome, early warning indicator
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