Abstract
Macrophage activation syndrome is a severe yet under-recognized complication encountered in pediatric rheumatology. It manifests as secondary hemophagocytic lymphohistiocytosis leading to a hyper-inflammatory state resulting from an underlying cytokine storm. If unchecked, it may lead to multiorgan failure and mortality. Early diagnosis and timely initiation of specific therapy is pivotal for a successful outcome. This review outlines the key clinical and laboratory features and management of macrophage activation syndrome.
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Contributors: NKB, AVR: conception of idea; NKB: reviewed the literature, prepared the initial draft; LGJES,AVR: reviewed the draft and revised it critically for important intellectual content; The final version of the manuscript have been critically revised and approved by all authors.
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Bagri, N.K., Gupta, L., Sen, E.S. et al. Macrophage Activation Syndrome in Children: Diagnosis and Management. Indian Pediatr 58, 1155–1161 (2021). https://doi.org/10.1007/s13312-021-2399-8
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DOI: https://doi.org/10.1007/s13312-021-2399-8