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14 900 ng/mL，LDH中位数为 1 264.5 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.