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Year : 2012  |  Volume : 7  |  Issue : 5  |  Page : 27-35

Macrophage activation syndrome

1 Universitą degli Studi di Genova, Genova, Italy
2 Universitą degli Studi di Genova, Genova; Istituto di Ricovero e Cura a Carattere Scientifico G. Gaslini, Genova, Italy
3 Istituto di Ricovero e Cura a Carattere Scientifico G. Gaslini, Genova, Italy

Correspondence Address:
Angelo Ravelli
Universitą degli Studi di Genova, Genova; Istituto di Ricovero e Cura a Carattere Scientifico G. Gaslini, Genova, Italy

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Source of Support: None, Conflict of Interest: None

DOI: 10.1016/S0973-3698(12)60026-0

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Macrophage activation syndrome (MAS) is a serious, potentially life-threatening complication of rheumatic disorders, which is seen most commonly in systemic juvenile idiopathic arthritis (sJIA). It is characterised clinically by unremitting high fever, pancytopaenia, hepatosplenomegaly, hepatic dysfunction, encephalopathy, coagulation abnormalities and sharply increased levels of ferritin. The pathognomonic feature of the syndrome is seen on bone marrow examina- tion, which frequently, though not always, reveals numerous morphologically benign macrophages exhibiting hae- mophagocytic activity. Macrophage activation syndrome is overt in 10% of children with sJIA but occurs subclinically in another 30-40%. Because MAS can follow a rapidly fatal course, prompt recognition of its clinical and laboratory features and immediate therapeutic intervention are essential. However, it is difficult to distinguish sJIA disease flare, infectious complications or medication side effects from MAS. A multinational collaborative effort aimed to develop diagnostic criteria for MAS in sJIA is under way. Although, the pathogenesis of MAS is unclear, the hallmark of the syn- drome is an uncontrolled activation and proliferation of T lymphocytes and macrophages, leading to massive hyper- secretion of pro-inflammatory cytokines. Mutations in cytolytic pathway genes are increasingly being recognised in children who develop MAS as part of sJIA. Recently, a mouse model of MAS dependent on repeated stimulation through toll-like receptors was developed. The first-line therapy of MAS complicating sJIA is based on the parenteral administration of high doses of corticosteroids, with or without cyclosporine A. There is increasing evidence that bio- logical therapies, particularly interleukin-1 inhibitors, represent a valuable adjunct to corticosteroids and cyclosporine A in treating MAS complicating sJIA.

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