Pathogenesis of macrophage activation syndrome and potential for cytokine- directed therapies.
Pathogenesis of macrophage activation syndrome and potential for cytokine- directed therapies.
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DOI：
10.1146/annurev-med-061813-012806
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发表时间：
2015
影响因子：
10.5
通讯作者：
Grom AA
中科院分区：
文献类型：
--
作者：
Schulert GS;Grom AA
巨噬细胞激活综合征 (MAS) 是一种严重炎症的急性发作，其特征是 T 淋巴细胞和噬血细胞巨噬细胞的激活和扩张。在风湿病学中，它最常发生在系统性幼年特发性关节炎 (SJIA) 和系统性红斑狼疮患者中。主要临床表现包括血细胞减少、肝功能障碍、类似弥散性血管内凝血的凝血病和极度高铁蛋白血症。在临床和病理学上，MAS 与噬血细胞性淋巴组织细胞增多症 (HLH) 非常相似，一些作者更喜欢用继发性 HLH 一词来描述它。其发病机制的核心是细胞因子风暴，包括 IL-1、IL-6、IL-18、TNFα 和 IFNγ 在内的多种促炎细胞因子水平显着升高。尽管有证据表明IFNγ可能在MAS的发病机制中发挥核心作用，但其他细胞因子的作用仍不清楚。有几份报告表明，SJIA 相关的 MAS 可以从阿那白滞素（一种重组 IL-1 受体拮抗剂）中显着受益，但其他生物制剂在 MAS 中的效用尚不清楚。主要治疗方法仍然是皮质类固醇；其他药物，包括环孢素，用于治疗无效的患者。
Macrophage activation syndrome (MAS) is an acute episode of overwhelming inflammation characterized by activation and expansion of T lymphocytes and hemophagocytic macrophages. In rheumatology, it occurs most frequently in patients with systemic juvenile idiopathic arthritis (SJIA) and systemic lupus erythematosus. The main clinical manifestations include cytopenias, liver dysfunction, coagulopathy resembling disseminated intravascular coagulation, and extreme hyperferritinemia. Clinically and pathologically, MAS bears strong similarity to hemophagocytic lymphohistiocytosis (HLH), and some authors prefer the term secondary HLH to describe it. Central to its pathogenesis is a cytokine storm, with markedly increased levels of numerous proinflammatory cytokines including IL-1, IL-6, IL-18, TNFα, and IFNγ. Although there is evidence that IFNγ may play a central role in the pathogenesis of MAS, the role of other cytokines is still not clear. There are several reports of SJIA-associated MAS dramatically benefiting from anakinra, a recombinant IL-1 receptor antagonist, but the utility of other biologics in MAS is not clear. The mainstay of treatment remains corticosteroids; other medications, including cyclosporine, are used in patients who fail to respond.
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影响因子：
158.5
作者：
De Benedetti, Fabrizio;Brunner, Hermine I.;Martini, Alberto
通讯作者：
Martini, Alberto
影响因子：
4.9
作者：
Bode SF;Lehmberg K;Maul-Pavicic A;Vraetz T;Janka G;Stadt UZ;Ehl S
通讯作者：
Ehl S
影响因子：
--
作者：
de Jager, Wilco;Vastert, Sebastiaan J.;Prakken, Berent J.
通讯作者：
Prakken, Berent J.
影响因子：
--
作者：
Gattorno, Marco;Piccini, Alessandra;Rubartelli, Anna
通讯作者：
Rubartelli, Anna
影响因子：
--
作者：
Fall, Ndate;Barnes, Michael;Grom, Alexei A.
通讯作者：
Grom, Alexei A.