ArticleBMJ case reports2025
Marked exacerbation of resolving macrophage activation syndrome in a patient with adult-onset Still's disease following one dose of granulocyte colony-stimulating factor.
Article in BMJ case reports, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
Macrophage activation syndrome (MAS) is a frequent complication of adult-onset Still's disease (AOSD) and is characterised by organ dysfunction, cytopenia and coagulopathy. There are few data regarding the use of granulocyte colony-stimulating factor (G-CSF) as a treatment for neutropenia in MAS due to AOSD. This case describes a previously healthy mid-20s patient who was diagnosed with AOSD and subsequently MAS. Although his clinical symptoms and ferritin levels responded well to steroids, interleukin-1 inhibition and Janus kinase inhibition, he developed prolonged and profound neutropenia for which he received one dose of G-CSF. His MAS flared markedly, requiring intensive immunosuppression and significantly prolonging his hospital stay. This report illustrates that G-CSF carries the risk of worsening inflammation leading to MAS, particularly in auto-inflammatory conditions such as AOSD.
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