CD8+ T Cells in Facioscapulohumeral Muscular Dystrophy Patients with Inflammatory Features at Muscle MRI
CD8+ T Cells in Facioscapulohumeral Muscular Dystrophy Patients with Inflammatory Features at Muscle MRI
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DOI：
10.1007/s10875-010-9474-6
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发表时间：
2011-04-01
影响因子：
9.1
通讯作者：
Ricci, Enzo
中科院分区：
文献类型：
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作者：
Frisullo, Giovanni;Frusciante, Roberto;Ricci, Enzo
面肩肱型肌营养不良症 (FSHD) 是一种遗传性疾病，尽管强烈建议，但炎症对其发病机制的影响尚未得到证实。在 FSHD 患者中，我们通过免疫组织化学发现肌肉中主要由 CD8(+) T 细胞组成的炎症浸润，在 T2 加权短 tau 反转恢复磁共振成像 (T2-STIR-MRI) 序列上显示出高信号特征。因此，我们评估了 T2-STIR-MRI 序列上有或没有肌肉显示高信号特征的患者和对照患者中循环激活免疫细胞的存在以及细胞因子的产生。与没有肌肉表现出高信号特征的患者和对照组相比，在一根或多块肌肉中表现出高信号特征的 FSHD 患者表现出更高的 CD8(+)pSTAT1(+)、CD8(+)T-bet(+) T 细胞和 CD14(+)pSTAT1(+)、CD14(+)T-bet(+) 细胞百分比以及 IL12p40、IFN γ 和 TNF α 水平。此外，CD8(+)pSTAT1(+)、CD8(+)T-bet(+)和CD14(+)pSTAT1(+)细胞的百分比与T2-STIR序列中表现出高信号特征的肌肉比例相关。这些数据表明，循环激活的免疫细胞（主要是 CD8+ T 细胞）可能通过促进肌肉炎症的活跃阶段来促进 FSHD 的进展。
Facioscapulohumeral muscular dystrophy (FSHD) is an inherited disease, and although strongly suggested, a contribution of inflammation to its pathogenesis has never been demonstrated. In FSHD patients, we found by immunohistochemistry inflammatory infiltrates mainly composed by CD8(+) T cells in muscles showing hyperintensity features on T2-weighted short tau inversion recovery magnetic resonance imaging (T2-STIR-MRI) sequences. Therefore, we evaluated the presence of circulating activated immune cells and the production of cytokines in patients with or without muscles showing hyperintensity features on T2-STIR-MRI sequences and from controls. FSHD patients displaying hyperintensity features in one or more muscles showed higher CD8(+)pSTAT1(+), CD8(+)T-bet(+) T cells and CD14(+)pSTAT1(+), CD14(+)T-bet(+) cells percentages and IL12p40, IFN gamma and TNF alpha levels than patients without muscles displaying hyperintense features and controls. Moreover, the percentages of CD8(+)pSTAT1(+), CD8(+)T-bet(+) and CD14(+)pSTAT1(+) cells correlated with the proportion of muscles displaying hyperintensity features at T2-STIR sequences. These data indicate that circulating activated immune cells, mainly CD8(+) T cells, may favour FSHD progression by promoting active phases of muscle inflammation.