{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,30]],"date-time":"2026-10-30T17:54:25Z","timestamp":1793382865927,"version":"4.5.0"},"reference-count":18,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2026,7,28]],"date-time":"2026-07-28T00:00:00Z","timestamp":1785196800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Pediatr."],"abstract":"<jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>To explore the diagnostic challenges of Kawasaki disease (KD)-associated macrophage activation syndrome (MAS) and the therapeutic value of ruxolitinib combined with corticosteroids.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>We retrospectively analyzed the clinical data of one pediatric patient with KD-associated MAS and conducted sorting and analysis of relevant published literature.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>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.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>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.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.3389\/fped.2026.1845017","type":"journal-article","created":{"date-parts":[[2026,7,28]],"date-time":"2026-07-28T05:38:03Z","timestamp":1785217083000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":1,"title":["Ruxolitinib combined with corticosteroids for the successful treatment of kawasaki disease-associated macrophage activation syndrome: a case report"],"prefix":"10.3389","volume":"14","author":[{"given":"Xuan","family":"Wang","sequence":"first","affiliation":[{"name":"Department of Endocrinology, The 940th Hospital of Joint Logistics Support Force of Chinese People\u2019s Liberation Army","place":["Lanzhou, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jiaofeng","family":"Bai","sequence":"additional","affiliation":[{"name":"Department of Hematology, The 940th Hospital of Joint Logistics Support Force of Chinese People\u2019s Liberation Army","place":["Lanzhou, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Huihui","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Hematology, The 940th Hospital of Joint Logistics Support Force of Chinese People\u2019s Liberation Army","place":["Lanzhou, China"]},{"name":"The First Clinical College of Gansu University of Traditional Chinese Medicine","place":["Lanzhou, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yanan","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Hematology, The 940th Hospital of Joint Logistics Support Force of Chinese People\u2019s Liberation Army","place":["Lanzhou, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mayire","family":"Ainiwaner","sequence":"additional","affiliation":[{"name":"Department of Hematology, The 940th Hospital of Joint Logistics Support Force of Chinese People\u2019s Liberation Army","place":["Lanzhou, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yaozhu","family":"Pan","sequence":"additional","affiliation":[{"name":"Department of Hematology, The 940th Hospital of Joint Logistics Support Force of Chinese People\u2019s Liberation Army","place":["Lanzhou, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2026,7,28]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"1641","DOI":"10.1007\/s00467-009-1336-1","article-title":"Medium-size-vessel vasculitis","volume":"25","author":"Dillon","year":"2010","journal-title":"Pediatr Nephrol"},{"key":"B2","doi-asserted-by":"publisher","first-page":"e927","DOI":"10.1161\/CIR.0000000000000484","article-title":"Diagnosis, treatment, and long-term management of kawasaki disease: a scientific statement for health professionals from the American Heart Association","volume":"135","author":"McCrindle","year":"2017","journal-title":"Circulation"},{"key":"B3","doi-asserted-by":"publisher","first-page":"566","DOI":"10.1007\/s12519-020-00360-6","article-title":"Macrophage activation syndrome in children with kawasaki disease: diagnostic and therapeutic approaches","volume":"16","author":"Han","year":"2020","journal-title":"World J Pediatr"},{"key":"B4","doi-asserted-by":"publisher","first-page":"124","DOI":"10.1002\/pbc.21039","article-title":"HLH-2004: diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis","volume":"48","author":"Henter","year":"2007","journal-title":"Pediatr Blood Cancer"},{"key":"B5","doi-asserted-by":"publisher","first-page":"1604648","DOI":"10.3389\/fimmu.2025.1604648","article-title":"Efficacy and safety of ruxolitinib in adult patients with refractory rheumatic disease-associated macrophage activation syndrome","volume":"16","author":"Li","year":"2025","journal-title":"Front Immunol"},{"key":"B6","doi-asserted-by":"publisher","first-page":"755","DOI":"10.3390\/children11070755","article-title":"Macrophage activation syndrome in children: update on diagnosis and treatment","volume":"11","author":"Lee","year":"2024","journal-title":"Children"},{"key":"B7","doi-asserted-by":"publisher","first-page":"349","DOI":"10.3390\/children12030349","article-title":"Macrophage activation syndrome in kawasaki disease: insights from a systematic literature review on diagnosis, clinical features, and treatment","volume":"12","author":"Inguscio","year":"2025","journal-title":"Children"},{"key":"B8","doi-asserted-by":"publisher","first-page":"105","DOI":"10.4078\/jrd.2024.0118","article-title":"Macrophage activation syndrome in kawasaki disease: a literature review of Korean studies","volume":"32","author":"Jeong","year":"2025","journal-title":"J Rheum Dis"},{"key":"B9","doi-asserted-by":"publisher","first-page":"94","DOI":"10.1080\/08998280.2024.2384042","article-title":"Breaking the storm: ruxolitinib as a lifeline in refractory hemophagocytic lymphohistiocytosis with macrophage activation syndrome","volume":"38","author":"Escobar Gil","year":"2025","journal-title":"Proc (Bayl Univ Med Cent)"},{"key":"B10","doi-asserted-by":"publisher","first-page":"612","DOI":"10.1182\/blood.2024024362","article-title":"Ruxolitinib targets JAK-STAT signaling to modulate neutrophil activation in refractory macrophage activation syndrome","volume":"146","author":"Ma","year":"2025","journal-title":"Blood"},{"key":"B11","doi-asserted-by":"publisher","first-page":"860","DOI":"10.1097\/CCM.0000000000005361","article-title":"Consensus-Based guidelines for the recognition, diagnosis, and management of hemophagocytic lymphohistiocytosis in critically ill children and adults","volume":"50","author":"Hines","year":"2022","journal-title":"Crit Care Med"},{"key":"B12","doi-asserted-by":"publisher","first-page":"206","DOI":"10.1182\/hematology.2025000707","article-title":"Hemophagocytic lymphohistiocytosis: do we have a solution for TMI (too much inflammation)?","volume":"2025","author":"La Ros\u00e9e","year":"2025","journal-title":"Hematology"},{"key":"B13","doi-asserted-by":"publisher","first-page":"1412","DOI":"10.1111\/1756-185X.13915","article-title":"Macrophage activation syndrome in pediatrics: 10 years data from an Indian center","volume":"23","author":"Pal","year":"2020","journal-title":"Int J Rheum Dis"},{"key":"B14","doi-asserted-by":"publisher","first-page":"672","DOI":"10.3390\/children9050672","article-title":"Efficacy of anakinra on multiple coronary arteries aneurysms in an infant with recurrent kawasaki disease, complicated by macrophage activation syndrome","volume":"9","author":"Bossi","year":"2022","journal-title":"Children"},{"key":"B15","doi-asserted-by":"publisher","first-page":"729","DOI":"10.1038\/nri.2017.129","article-title":"Immune editing shapes the cancer landscape","volume":"17","author":"Starling","year":"2017","journal-title":"Nat Rev Immunol"},{"key":"B16","doi-asserted-by":"publisher","first-page":"3493","DOI":"10.1182\/blood.2021014860","article-title":"A study of ruxolitinib response\u2013based stratified treatment for pediatric hemophagocytic lymphohistiocytosis","volume":"139","author":"Zhang","year":"2022","journal-title":"Blood"},{"key":"B17","doi-asserted-by":"publisher","first-page":"1769006","DOI":"10.3389\/fimmu.2026.1769006","article-title":"Accelerated response and reduced chemotherapy with ruxolitinib-based regimen therapy in pediatric hemophagocytic lymphohistiocytosis: a retrospective comparison with HLH-94 regimen","volume":"17","author":"Song","year":"2026","journal-title":"Front Immunol"},{"key":"B18","doi-asserted-by":"publisher","first-page":"1994","DOI":"10.1038\/s41598-017-18387-7","article-title":"Infliximab versus intravenous immunoglobulin for refractory kawasaki disease: a phase 3, randomized, open-label, active-controlled, parallel-group, multicenter trial","volume":"8","author":"Mori","year":"2018","journal-title":"Sci Rep"}],"container-title":["Frontiers in Pediatrics"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fped.2026.1845017\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,7,28]],"date-time":"2026-07-28T05:38:03Z","timestamp":1785217083000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fped.2026.1845017\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,7,28]]},"references-count":18,"alternative-id":["10.3389\/fped.2026.1845017"],"URL":"https:\/\/doi.org\/10.3389\/fped.2026.1845017","relation":{},"ISSN":["2296-2360"],"issn-type":[{"value":"2296-2360","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,7,28]]},"article-number":"1845017"}}