Macrophage activation syndrome in adults: recent advances in pathophysiology, diagnosis and treatment

Stuart J. Carter, Rachel S. Tattersall, Athimalaipet V. Ramanan

Research output: Contribution to journalArticle (Academic Journal)peer-review

40 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)5-17
Number of pages13
JournalRheumatology
Volume58
Issue number1
Early online date21 Feb 2018
DOIs
Publication statusPublished - Jan 2019

Keywords

  • Journal Article

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