Macrophage activation syndrome in a patient with systemic lupus erythematosus undergoing cyclophosphamide treatment: a case report
Published: July 17 2023
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All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.
Authors
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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Citations
Dhote R, Simon J, Papo T, Detournay B, Sailler L, Andre MH, et al. Reactive hemophagocytic syndrome in adult systemic disease: report of twenty-six cases and literature review. Arthritis Rheum 2003; 49: 633-9. DOI: https://doi.org/10.1002/art.11368
Ravelli A. Macrophage activation syndrome. Curr Opin Rheumatol 2002; 14: 548-52. DOI: https://doi.org/10.1097/00002281-200209000-00012
Lerkvaleekul B, Vilaiyuk S. Macrophage activation syndrome: early diagnosis is key. Open Access Rheumatol 2018; 10: 117-28. DOI: https://doi.org/10.2147/OARRR.S151013
Crayne CB, Albeituni S, Nichols KE, Cron RQ. The immunology of macrophage activation syndrome. Front Immunol 2019; 10: 119. DOI: https://doi.org/10.3389/fimmu.2019.00119
Hadchouel M, Prieur AM, Griscelli C. Acute hemorrhagic, hepatic, and neurologic manifestations in juvenile rheumatoid arthritis: possible relationship to drugs or infection. J Pediatr 1985; 106: 561-6. DOI: https://doi.org/10.1016/S0022-3476(85)80072-X
Egües Dubuc CA, Uriarte Ecenarro M, Meneses Villalba C, Aldasoro Cáceres V, Hernando Rubio I, Belzunegui Otano J. Hemophagocytic syndrome as the initial manifestation of systemic lupus erythematosus. Reumatol Clin 2014; 10: 321-4. DOI: https://doi.org/10.1016/j.reumae.2013.09.004
Granata G, Didona D, Stifano G, Feola A, Granata M. Macrophage activation syndrome as onset of systemic lupus erythematosus: a case report and a review of the literature. Case Rep Med 2015; 2015: 294041. DOI: https://doi.org/10.1155/2015/294041
Aziz A, Castaneda EE, Ahmad N, Veerapalli H, Rockferry AG, Lankala CR, et al. Exploring macrophage activation syndrome secondary to systemic lupus erythematosus in adults: a systematic review of the literature. Cureus 2021; 13: e18822. DOI: https://doi.org/10.7759/cureus.18822
Fardet L, Galicier L, Lambotte O, Marzac C, Aumont C, Chahwanet D, et al. Development and validation of the HScore, a score for the diagnosis of reactive hemophagocytic syndrome. Arthritis Rheumatol 2014; 66: 2613-20. DOI: https://doi.org/10.1002/art.38690
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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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