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Macrophage activation syndrome triggered by systemic lupus erythematosus flare: successful treatment with a combination of dexamethasone sodium phosphate, intravenous immunoglobulin, and cyclosporine: a case report
Gouda, Wesam Alsaqabi, Faisal Moshrif, Abdelhfeez Abbas, Awad S. Abdel-Aziz, Tarek M.
Gouda, Wesam
Alsaqabi, Faisal
Moshrif, Abdelhfeez
Abbas, Awad S.
Abdel-Aziz, Tarek M.
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2021-10-07
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Abstract
Background: Macrophage activation syndrome is classified as a secondary form of hemophagocytic lymphohistiocytosis. It is a hyperinflammatory complication observed to be comorbid with a variety of autoimmune diseases, including adult-onset Still’s disease and systemic juvenile idiopathic arthritis. Macrophage activation syndrome is less commonly detected in adult patients with systemic lupus erythematosus, which, if untreated, can be fatal, though determining the optimum treatment strategy is still a challenge. Case presentation: Herein, we report a case of macrophage activation syndrome in a 33-year-old Egyptian female as an unusual complication of a systemic lupus erythematosus flare in adult patients. Our patient was initially treated with a combination of intravenous methylprednisolone pulse therapy and intravenous immunoglobulin therapy, which was followed by a course of oral prednisolone and oral cyclosporine with little response. Switching from oral prednisone to intravenous dexamethasone sodium phosphate showed a more favorable clinical and biochemical response. Conclusion: Macrophage activation syndrome is less commonly detected in adult patients with systemic lupus erythematosus. Our case demonstrates that dexamethasone sodium phosphate can be a successful alternative treatment for patients with systemic lupus erythematosus complicated by macrophage activation syndrome in whom the response to pulse methylprednisolone was inadequate to manage their illness, proving to be remarkably effective in a relatively short time frame.
Citation
Gouda, W., Alsaqabi, F., Moshrif, A. et al. Macrophage activation syndrome triggered by systemic lupus erythematosus flare: successful treatment with a combination of dexamethasone sodium phosphate, intravenous immunoglobulin, and cyclosporine: a case report. Journal of Medical Case Reports 15, 497 (2021). https://doi.org/10.1186/s13256-021-03072-1
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34620236 (pubmed)
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Journal article
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en
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© 2021 The Authors. Published by BMC (Springer Nature). This is an open access article available under a Creative Commons licence. The published version can be accessed at the following link on the publisher’s website: https://doi.org/10.1186/s13256-021-03072-1
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1752-1947
EISSN
1752-1947
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Licence for published version: Creative Commons Attribution 4.0 International