Case Reports
17 July 2023
Vol. 75 No. 2 (2023)
https://doi.org/10.4081/reumatismo.2023.1597

Macrophage activation syndrome in a patient with systemic lupus erythematosus undergoing cyclophosphamide treatment: a case report

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Macrophage activation syndrome, systemic lupus erythematosus, hemophagocytic lymphohistiocytosis, infection
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Authors

L. Fioravante da Silveira
https://orcid.org/0009-0000-2902-0177
Health Science Center, Department of Clinical Medicine, Federal University of Santa Maria, Brazil.
A. Zago
Health Science Center, Department of Clinical Medicine, Federal University of Santa Maria, Brazil.
D. Zanchet
https://orcid.org/0000-0001-7391-3837
Health Science Center, Department of Clinical Medicine, Federal University of Santa Maria, Brazil.
D. Chemello
chemello.diego@gmail.com
https://orcid.org/0000-0003-2051-2321
Health Science Center, Department of Clinical Medicine, Federal University of Santa Maria; Department of Health Sciences, Federal University of Santa Maria , Brazil.

Macrophage activation syndrome (MAS) is a disorder related to hemophagocytic lymphohistiocytosis and is a life-threatening complication of rheumatic diseases. The diagnosis is challenging because MAS symptoms are quite similar to those of many active autoimmune diseases or severe sepsis. We describe the case of a female patient with systemic lupus erythematosus that presented with symptoms suggesting acute decompensation of autoimmune disease and sepsis. She was later diagnosed with MAS. Despite an aggressive immunosuppressive treatment, she developed a fatal outcome.

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Macrophage activation syndrome in a patient with systemic lupus erythematosus undergoing cyclophosphamide treatment: a case report. (2023). Reumatismo, 75(2). https://doi.org/10.4081/reumatismo.2023.1597
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