https://doi.org/10.4081/reumatismo.2023.1597
Macrophage activation syndrome in a patient with systemic lupus erythematosus undergoing cyclophosphamide treatment: a case report
Submitted: 20 May 2023
Accepted: 30 June 2023
Published: 17 July 2023
Accepted: 30 June 2023
Abstract Views: 940
PDF: 537
Publisher's note
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.
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.
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
Supporting Agencies
NoneHow to Cite
Fioravante da Silveira, L., Zago, A. ., Zanchet, D., & Chemello, D. (2023). Macrophage activation syndrome in a patient with systemic lupus erythematosus undergoing cyclophosphamide treatment: a case report. Reumatismo, 75(2). https://doi.org/10.4081/reumatismo.2023.1597
Copyright (c) 2023 the Author(s)
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
Authors who publish with this journal agree to the following terms:
- Authors retain copyright and grant the journal right of first publication with the work simultaneously licensed under a Creative Commons Attribution License that allows others to share the work with an acknowledgement of the work's authorship and initial publication in this journal.
- Authors are able to enter into separate, additional contractual arrangements for the non-exclusive distribution of the journal's published version of the work (e.g., post it to an institutional repository or publish it in a book), with an acknowledgement of its initial publication in this journal.
- Authors are permitted and encouraged to post their work online (e.g., in institutional repositories or on their website) prior to and during the submission process, as it can lead to productive exchanges, as well as earlier and greater citation of published work.
Similar Articles
- Y. Emad, T. Gheita, H. Darweesh, P. Klooster, R. Gamal, H. Fathi, N. El-Shaarawy, M. Gamil, M. Hawass, R.M. El-Refai, H. Al-Hanafi, S. Abd-Ellatif, A. Ismail, J. Rasker, Antibodies to extractable nuclear antigens (ENAS) in systemic lupus erythematosus patients: correlations with clinical manifestations and disease activity , Reumatismo: Vol. 70 No. 2 (2018)
- E. Cipriano, F. Ceccarelli, L. Massaro, F. R. Spinelli, C. Alessandri, C. Perricone, G. Valesini, F. Conti, Joint involvement in patients affected by systemic lupus erythematosus: application of the swollen to tender joint count ratio , Reumatismo: Vol. 67 No. 2 (2015)
- F. Dall'Ara, I. Cavazzana, M. Frassi, M. Taraborelli, M. Fredi, F. Franceschini, L. Andreoli, M. Rossi, C. Cattaneo, A. Tincani, P. Airò, Macrophage activation syndrome in adult systemic lupus erythematosus: report of seven adult cases from a single Italian rheumatology center , Reumatismo: Vol. 70 No. 2 (2018)
- A. Bontadi, A. Ruffatti, S Giannini, E. Falcinelli, M. Tonello, A. Hoxha, P. Gresele, L. Punzi, In vitro effect of anti-β2 glycoprotein I antibodies on P-selectin expression, a marker of platelet activation , Reumatismo: Vol. 64 No. 1 (2012)
- M. Fredi, M. Bianchi, L. Andreoli, G. Greco, I. Olivieri, S. Orcesi, E. Fazzi, C. Cereda, A. Tincani, Typing TREX1 gene in patients with systemic lupus erythematosus , Reumatismo: Vol. 67 No. 1 (2015)
- L. Iaccarino, A. Ghirardello, M. Zen, D. Villalta, A. Tincani, L. Punzi, A. Doria, Polarization of TH2 response is decreased during pregnancy in systemic lupus erythematosus , Reumatismo: Vol. 64 No. 5 (2012)
- D. Jain, H.K. Aggarwal, V. Kaverappa, S. Dhayia, P. Jain, S. Yadav, Anti-dsDNA negative and anti-Ro positive lupus nephritis: a report of a rare case , Reumatismo: Vol. 65 No. 6 (2013)
- M. A. Marie, R. E. Abu Khalil, H. M. Habib, Urinary CXCL10: a marker of nephritis in lupus patients , Reumatismo: Vol. 65 No. 6 (2013)
- G. Pasero, P. Marson, Short story of antirheumatic therapy.VIII. The immunodepressants , Reumatismo: Vol. 64 No. 1 (2012)
- M. Taraborelli, M.G. Lazzaroni, N. Martinazzi, M. Fredi, I. Cavazzana, F. Franceschini, A. Tincani, The role of clinically significant antiphospholipid antibodies in systemic lupus erythematosus , Reumatismo: Vol. 68 No. 3 (2016)
You may also start an advanced similarity search for this article.
