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Glucocorticoids (GCs) are the main treatment currently; however, 37.9% to 61% of patients fail to achieve effective control of HLH, making it urgent to find novel treatment strategies. We conducted a retrospective, single\u2010centre study examining ruxolitinib (RUX)\u2010based regimen in children with AD\/AID\u2010HLH. Patients were first treated with RUX monotherapy, and additional treatments including methylprednisolone and etoposide were added sequentially when the disease could not be controlled. The study included 26 patients with a median follow\u2010up of 23.9\u2009months, of whom 15 had prior treatments. The overall response rate at week 8 with the RUX\u2010based regimen was 96.2%, with 92.3% attaining complete response (CR) and 3.9% attaining partial response. The 2\u2010year overall survival rate was 96.2% (95% CI, 80.4% to 99.9%). During RUX monotherapy, 46.1% of patients achieved CR as the best response, with a median first response time to RUX of 2\u2009days. Additionally, 53.8% of patients required additional GCs and 23.1% required etoposide chemotherapy. All observed adverse events were manageable and acceptable. Overall, our study supports the efficacy and safety of the RUX\u2010based regimen in children with AD\/AID\u2010HLH.<\/jats:p>","DOI":"10.1111\/bjh.19803","type":"journal-article","created":{"date-parts":[[2024,10,10]],"date-time":"2024-10-10T12:53:52Z","timestamp":1728564832000},"page":"215-223","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["Ruxolitinib\u2010based regimen in children with autoimmune disease or autoinflammatory disease\u2010related haemophagocytic lymphohistiocytosis"],"prefix":"10.1111","volume":"206","author":[{"ORCID":"https:\/\/orcid.org\/0009-0005-2765-9635","authenticated-orcid":false,"given":"Zishi","family":"Fang","sequence":"first","affiliation":[{"name":"Hematological Department Beijing Children's Hospital, Capital Medical University, National Center for Children's Health  Beijing China"},{"name":"Hematologic Disease Laboratory Beijing Pediatric Research Institute, Beijing Children's 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