儿童全身型幼年特发性关节炎与系统性红斑狼疮合并巨噬细胞活化综合征临床特点对比研究

Comparative analysis of pediatric macrophage activation syndrome combined with systemic juvenile idiopathic arthritis versus with systemic lupus erythematosus

目的比较全身型幼年特发性关节炎(systemic juvenile idiopathic arthritis,sJIA)与幼年系统性红斑狼疮(juvenile onset systemic lupus erythematosus,JSLE)并发巨噬细胞活化综合征(macrophage activation syndrome,MAS)临床特点的异同。方法回顾性分析2015年5月至2023年1月河北省儿童医院肾脏免疫科收治的48例MAS患儿的病例资料,分为sJIA-MAS组及JSLE-MAS组,比较2组的临床表现、实验室指标及治疗情况。结果48例MAS患儿,男14例,女34例,发病年龄9.5(3.0,11.8)岁。sJIA-MAS 28例,男11例,女17例;JSLE-MAS 20例,男3例,女17例。48例MAS患儿均有发热和高铁蛋白血症,sJIA-MAS多为稽留热或弛张热。sJIA-MAS[15例(53.6%)]最常见的触发因素是呼吸道感染,JSLE-MAS[13例(65.0%)]最常见的触发因素是疾病活动。EB病毒与巨细胞病毒感染[sJIA 7例(25%),JSLE 4例(20%)]也是触发MAS因素之一。相比JSLE-MAS,sJIA-MAS发热天数[15.0(12.0,21.0)d比6.0(4.0,9.5)d,Z=-3.812,P=0.001]与住院天数[29.0(26.3,39.8)d比26.0(19.3,30.8)d,Z=-1.958,P=0.049]更长。相比JSLE-MAS,sJIA-MAS丙氨酸氨基转移酶[(685.32±561.67)U/L比(139.61±124.44)U/L,t=4.973,P=0.001]、天冬氨酸氨基转移酶[784.00(235.25,1251.25)U/L比189.50(53.25,374.08)U/L,Z=-3.283,P=0.001]、CRP[11.48(3.56,28.89)mg/L比1.91(0.53,8.98)mg/L,Z=-3.200,P=0.001]、铁蛋白[32167.0(12384.8,65963.8)μg/L比2003.5(922.5,11430.0)μg/L,Z=-4.130,P=0.001]、铁蛋白max/ESR min[1353.35(355.75,4342.53)比91.92(34.94,291.53),Z=-4.120,P=0.001]更高,CRP下降的幅度[80.04(45.64,143.71)mg/L比10.20(6.27,25.64)mg/L,Z=-4.433,P=0.001]更大。相比sJIA-MAS,JSLE-MAS外周血白细胞计数[4.05(2.90,7.73)×10^(9)/L比1.56(1.15,3.47)×10^(9)/L,Z=-3.577,P=0.001]、血小板计数[(162.68±92.19)×10^(9)/L比(110.10±72.99)×10^(9)/L,t=2.118,P=0.040]更低,JSLE-MAS肾脏受累更常见[10例(50%)比0例(0%),χ^(2)=17.684,P=0.001]。符合噬血细胞综合征标准的sJIA-MAS与JSLE-MAS发生率差异无统计学意义[6例(21.4%)比5例(25.0%),χ^(2)=0.084,P=0.772]。结论sJIA-MAS与JSLE-MAS相比,sJIA-MAS病情更凶险,不易控制;而JSLE-MAS脏器损害较多,血液系统、肾脏受累更常见。

Objective To compare the similarities and differences of macrophage activation syndrome(MAS)combined with systemic juvenile idiopathic arthritis(sJIA)versus with juvenile onset systemic lupus erythematosus(JSLE).Methods The clinical data of 48 children with MAS admitted to the Department of Nephrology and Immunology in Children's Hospital of Hebei Province from May 2015 to January 2023 were retrospectively analyzed.The patients were divided into sJIA-MAS and JSLE-MAS group,and the clinical manifestations,laboratory indicators and treatment of the two groups were compared.Results Among the 48 children(14 males and 34 females)with MAS,the average age of onset was 9.5(3.0,11.8)years.There were 28 cases(11males and 17 females)of sJIA-MAS and 20 cases(3 males and 17 females)of JSLE-MAS.All the 48 children with MAS had fever and hyperferinemia,and the fever with sJIA-MAS was mostly continued fever or remittent fever.Respiratory tract infection was the most common trigger in sJIA-MAS[15 cases(53.6%)],and disease activity was the most common trigger in JSLE-MAS[13 cases(65.0%)].Additionally,viral infections(EB virus and cytomegalovirus)were also one of the triggers in MAS[sJIA:7 cases(25%),JSLE:4 cases(20%)].Compared with JSLE-MAS,the number of days with fever[15.0(12.0,21.0)days vs.6.0(4.0,9.5)days,Z=-3.812,P=0.001]and the length of hospital stay[29.0(26.3,39.8)days vs.26.0(19.3,30.8)days,Z=-1.958,P=0.049]were longer in sJIA.Compared with JSLE-MAS,ALT[(685.32±561.67)U/L vs.(139.61±124.44)U/L,t=4.973,P=0.001],AST[784.00(235.25,1251.25)U/L vs.189.50(53.25,374.08)U/L,Z=-3.283,P=0.001],CRP[11.48(3.56,28.89)mg/L vs.1.91(0.53,8.98)mg/L,Z=-3.200,P=0.001],ferritin[32167.0(12384.8,65963.8)μg/L vs.2003.5(922.5,11430.0)μg/L,Z=-4.130,P=0.001],ferritin max/ESR min[1353.35(355.75,4342.53)vs.91.92(34.94,291.53),Z=-4.120,P=0.001]were higher in sJIA.The decrease of CRP was greater in sJIA[80.04(45.64,143.71)mg/L vs.10.20(6.27,25.64)mg/L,Z=-4.433,P=0.001].Compared with sJIA-MAS,peripheral white blood cell counting[4.05(2.90,7.73)×10^(9)/L vs.1.56(1.15,3.47)×10^(9)/L,Z=-3.577,P=0.001]and platelet counting[(162.68±92.19)×10^(9)/L vs.(110.10±72.99)×10^(9)/L,t=2.118,P=0.040]were lower in JSLE-MAS.Kidney involvement was more common in JSLE-MAS[10 cases(50%)vs.0 cases(0%),χ^(2)=17.684,P=0.001].There was no significant difference in the incidence of sJIA-MAS and JSLE-MAS meeting the criteria of hemophagocytic lymphohistiocytosis[6 cases(21.4%)vs.5 cases(25.0%),χ^(2)=0.084,P=0.772].Conclusion Compared with JSLE-MAS,sJIA-MAS is more dangerous and difficult to control,while JSLE-MAS involves more organs,among which the blood system and kidney are more common.