期刊
BEST PRACTICE & RESEARCH IN CLINICAL RHEUMATOLOGY
卷 28, 期 2, 页码 277-292出版社
ELSEVIER SCI LTD
DOI: 10.1016/j.berh.2014.03.002
关键词
Macrophage activation syndrome; Hemophagocytic lymphohistiocytosis; Systemic juvenile idiopathic arthritis; Still's disease; IL-1; IL-6; IL-18; IFN gamma
类别
资金
- Novartis
- Roche
- Amgen Fellowship Training Award from the Rheumatology Research Foundation
- NIH grants [NIH RO1-AR059049, NIH PO1-AR048929]
Macrophage activation syndrome (MAS) is an episode of overwhelming inflammation that occurs most commonly in children with systemic juvenile idiopathic arthritis (SJIA). It is characterized by expansion and activation of T lymphocytes and hemophagocytic macrophages and bears great similarity to hemophagocytic lymphohistiocytosis (HLH). This disorder has substantial morbidity and mortality, and there is frequently a delay in recognition and initiation of treatment. Here, we will review what is known about the pathogenesis of MAS and, in particular, its similarities to HLH. The development of MAS is characterized by a cytokine storm, with the elaboration of numerous pro-inflammatory cytokines. We will examine the evidence for various cytokines in the initiation and pathogenesis of MAS and discuss how new biologic therapies may alter the risk of MAS. Finally, we will review current treatment options for MAS and examine how cytokine-directed therapy could serve as novel treatment modalities. (C) 2014 Elsevier Ltd. All rights reserved.
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