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儿科风湿病：全身型幼年特发性关节炎中巨噬细胞活化综合征的诊断。

Paediatric rheumatic disease: Diagnosing macrophage activation syndrome in systemic JIA.

机构信息

Center for Molecular and Cellular Intervention, Department of Pediatric Rheumatology and Immunology, University Medical Center Utrecht, PO Box 85090, 3508 AB Utrecht, Netherlands.

出版信息

Nat Rev Rheumatol. 2014 Nov;10(11):640-2. doi: 10.1038/nrrheum.2014.143. Epub 2014 Sep 9.

DOI:10.1038/nrrheum.2014.143
PMID:25201384
Abstract

Macrophage activation syndrome (MAS) in systemic juvenile idiopathic arthritis (sJIA) is difficult to distinguish from active sJIA or sepsis. A collaborative effort scoring the performance of different clinical diagnostic criteria provides valuable guidance to clinicians, but challenges in the diagnosis of sJIA-related MAS remain.

摘要

巨噬细胞活化综合征（MAS）在全身型幼年特发性关节炎（sJIA）中难以与活动期 sJIA 或脓毒症相鉴别。协作式评分努力对不同临床诊断标准的性能进行评分，为临床医生提供了有价值的指导，但 sJIA 相关 MAS 的诊断仍然存在挑战。

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本文引用的文献

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Performance of current guidelines for diagnosis of macrophage activation syndrome complicating systemic juvenile idiopathic arthritis.现行指南在诊断系统性幼年特发性关节炎并发巨噬细胞活化综合征时的表现。
Arthritis Rheumatol. 2014 Oct;66(10):2871-80. doi: 10.1002/art.38769.
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Follistatin-like protein 1 and the ferritin/erythrocyte sedimentation rate ratio are potential biomarkers for dysregulated gene expression and macrophage activation syndrome in systemic juvenile idiopathic arthritis.卵泡抑素样蛋白 1 和铁蛋白/红细胞沉降率比值是系统性幼年特发性关节炎基因表达失调和巨噬细胞活化综合征的潜在生物标志物。
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Familial and acquired hemophagocytic lymphohistiocytosis.
家族性和获得性噬血细胞性淋巴组织细胞增生症。
Annu Rev Med. 2012;63:233-46. doi: 10.1146/annurev-med-041610-134208.
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Pathogenesis of systemic juvenile idiopathic arthritis: some answers, more questions.全身性幼年特发性关节炎的发病机制：部分答案，更多疑问。
Nat Rev Rheumatol. 2011 Jun 7;7(7):416-26. doi: 10.1038/nrrheum.2011.68.
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Distinct cytokine profiles of systemic-onset juvenile idiopathic arthritis-associated macrophage activation syndrome with particular emphasis on the role of interleukin-18 in its pathogenesis.特发性关节炎相关巨噬细胞活化综合征全身型与细胞因子谱的差异，尤其强调白细胞介素-18 在其发病机制中的作用。
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Defective phosphorylation of interleukin-18 receptor beta causes impaired natural killer cell function in systemic-onset juvenile idiopathic arthritis.白细胞介素-18受体β磷酸化缺陷导致全身型幼年特发性关节炎中自然杀伤细胞功能受损。
Arthritis Rheum. 2009 Sep;60(9):2782-93. doi: 10.1002/art.24750.
7
The myeloid-related proteins 8 and 14 complex, a novel ligand of toll-like receptor 4, and interleukin-1beta form a positive feedback mechanism in systemic-onset juvenile idiopathic arthritis.髓样相关蛋白8和14复合物，一种Toll样受体4的新型配体，与白细胞介素-1β在全身型幼年特发性关节炎中形成正反馈机制。
Arthritis Rheum. 2009 Mar;60(3):883-91. doi: 10.1002/art.24349.
8
The diagnostic significance of soluble CD163 and soluble interleukin-2 receptor alpha-chain in macrophage activation syndrome and untreated new-onset systemic juvenile idiopathic arthritis.可溶性CD163和可溶性白细胞介素-2受体α链在巨噬细胞活化综合征及未经治疗的新发系统性幼年特发性关节炎中的诊断意义
Arthritis Rheum. 2007 Mar;56(3):965-71. doi: 10.1002/art.22416.
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HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis.HLH-2004：噬血细胞性淋巴组织细胞增生症的诊断与治疗指南。
Pediatr Blood Cancer. 2007 Feb;48(2):124-31. doi: 10.1002/pbc.21039.
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Preliminary diagnostic guidelines for macrophage activation syndrome complicating systemic juvenile idiopathic arthritis.全身型幼年特发性关节炎合并巨噬细胞活化综合征的初步诊断指南
J Pediatr. 2005 May;146(5):598-604. doi: 10.1016/j.jpeds.2004.12.016.