Abstract:

Objective  To analyze the clinical features of Kawasaki disease (KD) with onset of macrophage activation syndrome (MAS), and to discuss the diagnosis and treatment of KD complicated with MAS. Methods  The clinical data of two children with KD with onset of MAS in Children's Hospital Affiliated to Shandong University were retrospectively analyzed, and the relevant literatures were reviewed. Results  Child 1, boy, 3 years and 10 months old, was admitted to our hospital on March 8, 2022. With the onset of fever and rash, he had persistent hyperthermia after admission, accompanied by systemic rash, lactate dehydrogenase and aspartate transaminase (AST) progressive elevation. The child was diagnosed with KD complicated by MAS and was given intravenous immunoglobulin (IVIG) twice, followed by methylprednisolone shock therapy for 3 days, and treatment was sequentially discontinued for 1 month. The child's body temperature dropped to normal, and the rash gradually subsided. Child 2, girl, 1 year and 2 months old, was admitted to our hospital on July 27, 2022. With fever and anemia as the first symptoms, she was complicated by thrombocytopenia, fibrinogen reduction, and elevated ferritin. She was given IVIG twice, and followed by intravenous methylprednisolone for 11 days and oral methylprednisolone for 14 days. The child's body temperature dropped to normal, anemia was corrected, and platelets and ferritin returned to normal. Conclusions  The prognosis in children with KD with onset of MAS is good when they are actively treated. At present, there are no guidelines for the treatment of this disease, which requires long-term clinical observation and the establishment of more refined diagnostic criteria and treatment plans.

Key words:

Kawasaki disease, Macrophage activation syndrome, Clinical features, Immunoglobulin

摘要：

目的　分析以巨噬细胞活化综合征（MAS）起病的川崎病的临床特点，探讨川崎病合并巨噬细胞活化综合征的诊断及治疗方法。方法　回顾分析山东大学附属儿童医院2例以MAS起病的川崎病患儿的临床资料，并进行文献分析。结果　患儿1，男，3岁10个月，2022年3月8日于山东大学附属儿童医院入院治疗，以发热、皮疹起病，入院后持续高热，伴全身皮疹，乳酸脱氢酶、天冬氨酸转氨酶进行性升高，诊断川崎病并发MAS，给予2次静脉注射人免疫球蛋白（IVIG），并甲泼尼龙冲击治疗3 d后序贯治疗1个月减停，患儿体温降至正常，皮疹逐渐消退；患儿2，女，1岁2个月，2022年7月27日入院，以发热、贫血为首发症状，并发血小板减少、纤维蛋白原降低及铁蛋白升高，给予2次IVIG，并静脉滴注甲泼尼龙11 d及口服甲泼尼龙14 d治疗，患儿体温降至正常，贫血纠正，血小板、铁蛋白等恢复正常。结论　MAS起病的川崎病经积极治疗，患儿预后良好，目前没有针对此病治疗的诊治指南，需要长期临床观察，建立更精细的诊断标准及治疗方案。

关键词:

川崎病, 巨噬细胞活化综合征, 临床特点, 免疫球蛋白