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Letter
Ruxolitinib as a salvage therapy in adult-onset macrophage activation syndrome: insights from eight cases
  1. Ziyi Song1,2,
  2. Haihong Yao1,2,
  3. Yuebo Jin1,2,
  4. Xue Li1,2,
  5. Yuan Jia1,2,
  6. Jing He1,2,
  7. Zhanguo Li1,2,3
  1. 1 Department of Rheumatology and Immunology, Peking University People's Hospital, Beijing, People's Republic of China
  2. 2 Beijing Key Laboratory for Rheumatism Mechanism and Immune Diagnosis (BZ0135), Peking University People's Hospital, Beijing, People's Republic of China
  3. 3 Peking-Tsinghua Center for Life Sciences, Peking University People's Hospital, Beijing, People's Republic of China
  1. Correspondence to Dr Haihong Yao; yaohaihong{at}pku.edu.cn

https://doi.org/10.1136/ard-2024-226433

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    Macrophage activation syndrome (MAS) is a life-threatening complication of rheumatic disorder characteristic by excessive production of proinflammatory cytokines. In adults, MAS poses a major clinical challenge due to its rapid progression and high mortality rates. Current first-line treatment regimens, based on paediatric approaches, including haemophagocytic lymphohistiocytosis (HLH)-94 or HLH-2004, do not work for about 30% of adult patients. This unmet need highlights the urgency for targeted therapies and salvage treatments. Janus kinase inhibitors, like ruxolitinib (Rux), are emerging as promising options due to their capacity to selectively block proinflammatory cytokine pathways, offering a new therapeutic strategy for managing MAS in adults.1 2 Previous cases have reported Rux to be a safe and effective therapy, especially in refractory or relapsed HLH, including MAS. …

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    Footnotes

    • Handling editor Josef S Smolen

    • Contributors All authors participated in the topic of the discussion of this letter and agreed on the final manuscript. ZS is the guarantor.

    • Funding This study was funded by the National Key Research and Development Program of China (Grant/Award No 2022YFC3602000); the National Natural Science Foundation of China (Grant/Award No 81801618); and the Clinical Medicine Plus X-Young Scholars Project of Peking University (PKU2021LCXQ008) supported by the Fundamental Research Funds for the Central Universities.

    • Competing interests None declared.

    • Patient and public involvement Patients and/or the public were not involved in the design, or conduct, or reporting, or dissemination plans of this research.

    • Provenance and peer review Not commissioned; externally peer reviewed.