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首页> 外文期刊>Clinical immunology: The official journal of the Clinical Immunology Society >Distinct cytokine profile in juvenile systemic lupus erythematosus-associated macrophage activation syndrome
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Distinct cytokine profile in juvenile systemic lupus erythematosus-associated macrophage activation syndrome

机译：幼年系统性红斑狼疮相关巨噬细胞活化综合征的不同细胞因子谱

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Macrophage activation syndrome (MAS) has been observed in patients with systemic lupus erythematosus (SLE). Recognition of MAS in patients with SLE may be particularly challenging because it may mimic the clinical features of the underlying disease or be confused with an infectious complication. Massive hypercytokinemia is strongly associated with the pathogenesis of systemic lupus erythematosus-associated macrophage activation syndrome (SLE-MAS) but the pathogenesis and kinetics of cytokine release in SLE-MAS patients is not well studied. We present a case of SLE-MAS. The patient showed the distinct cytokine profile of SLE-MAS compared to systemic juvenile idiopathic arthritis associated MAS and Epstein-Barr virus-induced hemophagocytic lymphohistiocytosis. The observed TNF-?? dominant increase appears to be characteristic of SLE-MAS. IgM type antilymphocyte antibody (ALAB) was detected on the surface of lymphocytes during the acute phase and disappeared when the patient was in remission. The patient had a heterozygous P369S-R408Q mutation in the MEFV gene. Our results suggest that ALAB and a MEFV mutation might play important roles in the pathogenesis of SLE-MAS. Furthermore, the cytokine profile of SLE-MAS differs from that of S-JIA-MAS: the TNF-?? dominant increase appears to be characteristic. ? 2012 Elsevier Inc.
机译：系统性红斑狼疮（SLE）患者中已观察到巨噬细胞活化综合征（MAS）。在SLE患者中识别MAS可能特别具有挑战性，因为它可能模仿潜在疾病的临床特征或与感染性并发症相混淆。大规模高细胞血症与系统性红斑狼疮相关巨噬细胞活化综合征（SLE-MAS）的发病机理密切相关，但尚未对SLE-MAS患者的细胞因子释放的发病机理和动力学进行深入研究。我们介绍一个SLE-MAS案例。与系统性幼年特发性关节炎相关的MAS和爱泼斯坦-巴尔病毒引起的吞噬性淋巴细胞组织细胞增生相比，该患者显示出SLE-MAS独特的细胞因子谱。观察到的TNF-α显着增加似乎是SLE-MAS的特征。急性期在淋巴细胞表面检测到IgM型抗淋巴细胞抗体（ALAB），当患者缓解后消失。该患者的MEFV基因具有杂合的P369S-R408Q突变。我们的结果表明，ALAB和MEFV突变可能在SLE-MAS的发病机理中起重要作用。此外，SLE-MAS的细胞因子谱不同于S-JIA-MAS的细胞因子谱：TNF-α。显性增加似乎是特征。 ？ 2012爱思唯尔公司

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