title: Macrophage Activation Syndrome.
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.
authors.affiliation: 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.
authors.forename: Angelo
authors.initials: A
authors.lastname: Ravelli
authors.affiliation: Second Pediatric Division and Rheumatology, G. Gaslini Institute, via G. Gaslini 5, Genoa 16147, Italy.
authors.forename: Sergio
authors.initials: S
authors.lastname: Davì
authors.affiliation: Second Pediatric Division and Rheumatology, G. Gaslini Institute, via G. Gaslini 5, Genoa 16147, Italy.
authors.forename: Francesca
authors.initials: F
authors.lastname: Minoia
authors.affiliation: Department of Pediatrics and Second Pediatric Division and Rheumatology, University of Genoa and G. Gaslini Institute, via G. Gaslini 5, Genoa 16147, Italy.
authors.forename: Alberto
authors.initials: A
authors.lastname: Martini
authors.affiliation: 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.
authors.forename: Randy Q
authors.initials: RQ
authors.lastname: Cron
mesh_terms: D019468:Disease Management; D006801:Humans; D055501:Macrophage Activation Syndrome / Q000175:diagnosis* / Q000453:epidemiology / Q000209:etiology* / Q000628:therapy; D017410:Practice Guidelines as Topic; D012307:Risk Factors
keywords: Hemophagocytic lymphohistiocytosis; Hemophagocytic syndrome; Interleukin-1 inhibitors; Macrophage activation syndrome; Proinflammatory cytokines; Systemic juvenile idiopathic arthritis