川崎病与幼年特发性关节炎全身型合并巨噬细胞活化综合征临床特点对比分析  

Comparative analysis of clinical characteristics between Kawasaki disease and systemic macrophage activation syndrome and systematic-onset juvenile idiopathic arthritis

作　　者：周蔚然 刘雪梅[1] 董琳琳[1] 王兴翠[1] 潘艳艳[1] 王雪[1] 张洪霞[1] ZHOU Weiran;LIU Xuemei;DONG Linlin;WANG Xingcui;PAN Yanyan;WANG Xue;ZHANG Hongxia(Jinan Children’s Hospital,Jinan 250022,Shandong,China)

机构地区：[1]济南市儿童医院,山东济南250022

出　　处：《临床儿科杂志》2020年第10期744-748,共5页Journal of Clinical Pediatrics

基　　金：山东省重点研发计划(No.2017G00604)。

摘　　要：目的对比川崎病(KD)与幼年特发性关节炎全身型(SJIA)合并巨噬细胞活化综合征(MAS)的临床特征。方法回顾性分析2015年9月至2019年9月收治的5例KD-MAS及8例SJIA-MAS患儿的临床资料。结果KD-MAS患儿均为男性,年龄5个月~7岁10个月;SJIA-MAS组8例共10次MAS过程,男性3例、女性5例,年龄1岁2个月~11岁,其中男女各1例发生2次MAS,复发时年龄分别为6岁7个月、8岁5个月。所有患儿均以持续高热为主要临床表现;实验室指标比较,铁蛋白水平与LDH水平相关(P=0.03,r=0.071)。SJIA-MAS组铁蛋白中位数为14900 ng/mL,LDH中位数为1264.5 U/L;KD-MAS组铁蛋白中位数为736 ng/mL,LDH中位数为492 U/L;两组比较差异有统计学意义(P<0.05)。KDMAS组中1例、SJIA-MAS组中3例行基因检测,未检出HLH相关基因。SJIA-MAS组8例患儿MAS过程中均给予甲基泼尼松龙冲击加环孢素A治疗,2例复发,2例最终死亡。KD-MAS组患儿病初均给予2 g/kg免疫球蛋白治疗,诊断MAS后1例给予甲基泼尼松龙冲击治疗,1例给予甲基泼尼松龙冲击、环孢素及血浆置换治疗,其余3例给予甲基泼尼松龙7 mg/(kg·d),后逐渐减量;所有患儿均能够有效控制病情。KD-MAS组中3例符合HLH-2004诊断标准,5例符合SJIA-MAS 2016诊断标准。结论LDH可能作为除铁蛋白外另一个早期识别MAS的敏感指标,KD-MAS与SJIA-MAS铁蛋白、LDH水平上有差异,应用SJIA-MAS 2016诊断标准诊断KD-MAS的敏感性更高,KD-MAS治疗通过糖皮质激素即可达到较好疗效,且预后较SJIA-MAS更好。Objective To explore the comparison of the clinical features between Kawasaki disease(KD)and systematiconset juvenile idiopathic arthritis(SJIA)with macrophage activation syndrome(MAS).Method The clinical data of 5 cases of KD-MAS and 8 cases of SJIA-MAS children admitted from September 2015 to September 2019 were retrospectively analyzed.Results The children with KD-MAS were all male,aged from 5 months to 7 years and 10 months.In the SJIA-MAS group,8 children(3 boys and 5 girls)aged from 1 year and 2 months to 11 years had 10 times of MAS in total,among whom,one boy and one girl had two times of MAS respectively,and the age at recurrence was 6 years and 7 months,and 8 years and 5 months,respectively.The main clinical manifestations of all the children were persistent high fever.Comparison of laboratory indicators showed that ferritin level was correlated with LDH level(r=0.071,P=0.03).The median ferritin was 14900 ng/mL and the median LDH was 1264.5 U/L in the SJIA-MAS group.The median ferritin and LDH in the KD-MAS group was 736 ng/ml and 492 U/L respectively,and the differences between the two groups were statistically significant(both P<0.05).One case in the KD-MAS group and 3 cases in the SJIA-MAS group underwent genetic testing,and no HLH-related genes were detected.The condition of all children were able to be effectively controlled.Conclusion LDH may serve as another sensitive indicator for early identification of MAS in addition to ferritin.There are differences between KD-MAS and SJIA-MAS in ferritin and LDH levels.The application of SJIA-MAS 2016 diagnostic criteria to diagnose KD-MAS is more sensitive.KD-MAS can achieve better curative effect through glucocorticoid treatment,and the prognosis is better than SJIA-MAS.

关 键 词：巨噬细胞活化综合征 川崎病 幼年特发性关节炎全身型 铁蛋白 乳酸脱氢酶 

分 类 号：R725.4[医药卫生—儿科] R725.9[医药卫生—临床医学]