Fever of Unknown Cause: A Case of Macrophage Activation Syndrome

Macrophage Activation Syndrome

Authors

DOI:

https://doi.org/10.5281/zenodo.15569678

Keywords:

Fever of Unknown Origin, Macrophage Activation Syndrome, Child

Abstract

Fever of unknown origin (FUO) in children is a challenging diagnostic problem, as it can be caused by a wide range of infectious, inflammatory, malignant, or genetic disorders. We present the case of a 12-year-old girl with persistent fever, arthralgia, and elevated inflammatory markers. Despite extensive infectious, oncological, and rheumatologic investigations, no underlying cause was identified. Laboratory findings revealed markedly increased C-reactive protein, ferritin, and D-dimer levels, and mild hepatomegaly. Macrophage activation syndrome (MAS) was suspected due to persistent fever and hyperferritinemia, and confirmed by bone marrow aspiration. The patient responded rapidly to intravenous immunoglobulin and high-dose corticosteroids. This case emphasizes the importance of considering MAS in pediatric FUO and highlights the need for early recognition and treatment.

Author Biography

Pelin Arslan, İnönü University, Schoof of Medicine, Malatya, Türkiye

inönü üniversitesi çocuk alanında acil yandal öğrencisiyim uzman pediatristim

References

PETERSDORF RG, BEESON PB. Fever of unexplained origin: report on 100 cases. Medicine (Baltimore). 1961;40:1-30. doi:10.1097/00005792-196102000-00001

Durack DT, Street AC. Fever of unknown origin--reexamined and redefined. Curr Clin Top Infect Dis. 1991;11:35-51.

Cunha BA. Fever of unknown origin: clinical overview of classic and current concepts [published correction appears in Infect Dis Clin North Am. 2008 Jun;22(2):xv]. Infect Dis Clin North Am. 2007;21(4):867-vii. doi:10.1016/j.idc.2007.09.002

Hayakawa K, Ramasamy B, Chandrasekar PH. Fever of unknown origin: an evidence-based review. Am J Med Sci. 2012;344(4):307-316. doi:10.1097/MAJ.0b013e31824ae504

Wakai S, Murayama S, Gomi H. Diagnostic strategy for pediatric fever of unknown origin. Pediatr Int. 2022;64(1):e14911.2

Ravelli A, Minoia F, Davi S, et al. Macrophage activation syndrome. Nat Rev Rheumatol. 2012;8(5):301-309.

Grom AA, Horne A, De Benedetti F. Macrophage activation syndrome in the era of biologic therapy. Nat Rev Rheumatol. 2016;12(5):259-268. doi:10.1038/nrrheum.2015.179

Schulert GS, Grom AA. Pathogenesis of macrophage activation syndrome. Rheum Dis Clin North Am. 2015;41(4):537-546.

Grom AA, Jordan MB, Newburger PE, et al. Macrophage activation syndrome in the setting of pediatric rheumatologic disease. Arthritis Rheum. 2003;48(3):771-781

Schulert GS, Grom AA, Rosenbaum JT. Macrophage activation syndrome: a hemophagocytic syndrome associated with rheumatic diseases. J Rheumatol. 2014;41(6):1041-1048.

Yao TC, Lee JH, Yeh KW, et al. Treatment of macrophage activation syndrome in children with systemic juvenile idiopathic arthritis. Semin Arthritis Rheum. 2015;45(2):188-195.

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Published

2025-06-01

How to Cite

Arslan, P., & Karakuş, G. (2025). Fever of Unknown Cause: A Case of Macrophage Activation Syndrome: Macrophage Activation Syndrome. Avicenna Anatolian Journal of Medicine, 2(1), 15–17. https://doi.org/10.5281/zenodo.15569678

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Case Report

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