Macrophage Activation Syndrome

Angelo Ravelli; Sergio Davì; Francesca Minoia; Alberto Martini; Randy Q Cron

doi:10.1016/j.hoc.2015.06.010

Macrophage Activation Syndrome

Hematol Oncol Clin North Am. 2015 Oct;29(5):927-41. doi: 10.1016/j.hoc.2015.06.010. Epub 2015 Aug 25.

Authors

Angelo Ravelli¹, Sergio Davì², Francesca Minoia², Alberto Martini³, Randy Q Cron⁴

Affiliations

¹ Department of Neuroscience, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health, Head, Center of Rheumatology, University of Genoa and G. Gaslini Institute, via G. Gaslini 5, Genoa 16147, Italy. Electronic address: angeloravelli@ospedale-gaslini.ge.it.
² Second Pediatric Division and Rheumatology, G. Gaslini Institute, via G. Gaslini 5, Genoa 16147, Italy.
³ Department of Pediatrics and Second Pediatric Division and Rheumatology, University of Genoa and G. Gaslini Institute, via G. Gaslini 5, Genoa 16147, Italy.
⁴ Director, Division of Pediatric Rheumatology, Children's Hospital of Alabama and University of Alabama at Birmingham, Children's Park Place, Ste. 210 1601 4th Avenue South Birmingham, AL 35233, USA.

PMID: 26461152
DOI: 10.1016/j.hoc.2015.06.010

Abstract

Macrophage activation syndrome (MAS) is a potentially life-threatening complication of rheumatic disorders that occurs most commonly in systemic juvenile idiopathic arthritis. In recent years, there have been several advances in the understanding of the pathophysiology of MAS. Furthermore, new classification criteria have been developed. Although the place of cytokine blockers in the management of MAS is still unclear, interleukin-1 inhibitors represent a promising adjunctive therapy, particularly in refractory cases.

Keywords: Hemophagocytic lymphohistiocytosis; Hemophagocytic syndrome; Interleukin-1 inhibitors; Macrophage activation syndrome; Proinflammatory cytokines; Systemic juvenile idiopathic arthritis.

Publication types

Review

MeSH terms

Disease Management
Humans
Macrophage Activation Syndrome / diagnosis*
Macrophage Activation Syndrome / epidemiology
Macrophage Activation Syndrome / etiology*
Macrophage Activation Syndrome / therapy
Practice Guidelines as Topic
Risk Factors