Macrophage activation syndrome in adults: recent advances in pathophysiology, diagnosis and treatment
Top Cited Papers
- 21 February 2018
- journal article
- review article
- Published by Oxford University Press (OUP) in Rheumatology
- Vol. 58 (1), 5-17
- https://doi.org/10.1093/rheumatology/key006
Abstract
Haemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome, which if not promptly treated, can lead rapidly to critical illness and death. HLH is termed macrophage activation syndrome (MAS) when associated with rheumatic disease (where it is best characterized in systemic JIA) and secondary HLH (sHLH) when associated with other triggers including malignancy and infection. MAS/sHLH is rare and coupled with its mimicry of other conditions, is underrecognized. These inherent challenges can lead to diagnostic and management challenges in multiple medical specialties including haematology, infectious diseases, critical care and rheumatology. In this review we highlight the pathogenesis of MAS/sHLH including its underlying triggers, key clinical features and diagnostic challenges, prognostic factors and current treatments in adults.Keywords
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