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. 2021 Mar 26;58(12):1155–1161. doi: 10.1007/s13312-021-2399-8

Macrophage Activation Syndrome in Children: Diagnosis and Management

Narendra Kumar Bagri 1,✉, Latika Gupta 2, Ethan S Sen 3, A V Ramanan 4
PMCID: PMC8732159  PMID: 33772536

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.

Keywords: Hyper-inflammation, Secondary hemophagocytic lymphohistiocytosis, Juvenile idiopathic arthritis

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.

Funding: None

Competing interest: None stated.

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