Comparative analysis of pediatric macrophage activation syndrome combined with systemic juvenile idiopathic arthritis versus with systemic lupus erythematosus
Objective To compare the similarities and differences of macrophage activation syndrome(MAS)combined with systemic juvenile idiopathic arthritis(s JI A)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(235.25,1 251.25)U/Lvs.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[32 167.0(12 384.8,65 963.8)μg/L vs.2 003.5(922.5,11 430.0)µg/L,Z=-4.130,P=0.001],ferritinmax/ESRmin[1 353.35(355.75,4 342.53)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)×109/L vs.1.56(1.15,3.47)×109/L,Z=-3.577,P=0.001]and platelet counting[(162.68±92.19)×109/L vs.(110.10±72.99)×109/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％),x2=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％),x2=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.
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