文献类型：期刊文献

英文题名：Ruxolitinib combined with corticosteroids for the successful treatment of kawasaki disease-associated macrophage activation syndrome: a case report

作者：Wang, Xuan[1];Bai, Jiaofeng[2];Wang, Huihui[2,3];Wang, Yanan[2];Ainiwaner, Mayire[2];Pan, Yaozhu[2]

第一作者：Wang, Xuan

通信作者：Pan, YZ[1]

机构：[1]940th Hosp Joint Logist Support Force Chinese Peop, Dept Endocrinol, Lanzhou, Peoples R China;[2]940th Hosp Joint Logist Support Force Chinese Peop, Dept Hematol, Lanzhou, Peoples R China;[3]Gansu Univ Tradit Chinese Med, Clin Coll 1, Lanzhou, Peoples R China

第一机构：940th Hosp Joint Logist Support Force Chinese Peop, Dept Endocrinol, Lanzhou, Peoples R China

通信机构：[1]corresponding author), 940th Hosp Joint Logist Support Force Chinese Peop, Dept Hematol, Lanzhou, Peoples R China.

年份：2026

卷号：14

外文期刊名：FRONTIERS IN PEDIATRICS

收录：;Scopus(收录号：2-s2.0-105047135856);WOS:【SCI-EXPANDED(收录号:WOS:001845028100001)】；

基金：The author(s) declared that financial support was received for this work and/or its publication. This study was supported by the Gansu Provincial Key R&D Programme Projects (No. 25YFFA065) and Lanzhou Municipal Science and Technology Program Project (No. 2024-9-162).

语种：英文

外文关键词：corticosteroids; hemophagocytic lymphohistiocytosis; kawasaki disease; macrophage activation syndrome; pediatric; ruxolitinib

摘要：Objective To explore the diagnostic challenges of Kawasaki disease (KD)-associated macrophage activation syndrome (MAS) and the therapeutic value of ruxolitinib combined with corticosteroids.Methods We retrospectively analyzed the clinical data of one pediatric patient with KD-associated MAS and conducted sorting and analysis of relevant published literature.Results A 14-month-old male infant presented with fever and rash and was diagnosed with severe KD. Despite initial treatment with intravenous immunoglobulin (IVIG) and aspirin, he developed persistent fever, progressive cytopenia, hepatosplenomegaly, hypofibrinogenemia, hyperferritinemia, reduced NK-cell activity, and extremely elevated soluble interleukin-2 receptor (sCD25), fulfilling the HLH-2004 criteria for MAS. He was successfully treated with ruxolitinib plus methylprednisolone, with rapid resolution of fever, recovery of blood counts, and improvement in coagulation and inflammatory markers.Conclusions KD-associated MAS is rare but life-threatening. Early recognition of warning signs and prompt initiation of ruxolitinib-based combination therapy can effectively control cytokine storm and improve clinical outcomes.