Article Text
Summary
Macrophage activation syndrome (MAS) is a rare disease characterised by aberrant immune hyperactivation of T lymphocytes and macrophages driven by cytokine dysfunction. The HLH-2004 protocol is commonly used for the treatment of MAS, but significant toxicities are associated. We describe a case of MAS secondary to systemic lupus erythematosus in a young female that responded well to rituximab in lieu of etoposide. She continues to be in remission 1 year following the completion of rituximab infusion and is maintained on hydroxychloroquine. This case highlights the need for further research on the use of rituximab and other available biologics in the setting of MAS in order to help guide further alternative treatment decisions.
- systemic lupus erythematosus
- biological agents
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Footnotes
Contributors ZJ drafted the article and agrees to be accountable for the article and to ensure that all questions regarding the accuracy or integrity of the article are investigated and resolved. RS analysed and interpreted the data and revised the manuscript critically for important intellectual content. CT and MK analysed and interpreted the data and revised the manuscript critically for important intellectual content as well as providing final approval of the version published.
Competing interests None declared.
Patient consent Obtained.
Provenance and peer review Not commissioned; externally peer reviewed.