Skewed T-Cell Receptor Variable Beta Repertoire and Massive T-Cell Activation in Idiopathic Orofacial Granulomatosis

Skewed T-Cell Receptor Variable Beta Repertoire and Massive T-Cell Activation in Idiopathic Orofacial Granulomatosis
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特发性口面部肉芽肿病中 T 细胞受体变异 β 库和大量 T 细胞激活

DOI:
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发表时间:
2012
影响因子:
3.5
通讯作者:
M. Pierdominici
M. Pierdominici
中科院分区:
医学4区
文献类型:
--
作者:
A. Giovannetti;F. Mazzetta;A. Cavani;D. Pennino;E. Caprini;E. Ortona;G. Donato;E. Rosato;F. Salsano;M. Pierdominici

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口腔面部肉芽肿病(OFG)是一种临床病理实体,描述非干酪化肉芽肿的口腔病变,包括一系列疾病,如Melkersson-Rosenthal综合征。异常t细胞反应参与了OFG的发病机制,尽管目前关于这一问题的数据很少。在一名OFG患者中,我们观察到病灶水平和循环中几乎完整的CD4和CD8 t细胞受体(TCR) β链可变区(BV)库。然而,在CD4和更大程度上在CD8亚群中发现了寡克隆谱。这些发现与大量外周T细胞活化、初始T细胞减少、胸腺输出减少、细胞因子产生改变和细胞凋亡增加有关。我们的数据表明,T细胞在炎症部位随机涌入,反驳了主要过敏原在口腔粘膜水平起作用的假设。外周t细胞区室的严重失调表明OFG应被视为一种局部表现的全身性疾病。
Orofacial granulomatosis (OFG) is a clinicopathologic entity describing oral lesions with noncaseating granulomas including a spectrum of diseases such as the Melkersson-Rosenthal syndrome. The involvement of abnormal T-cell responses has been suggested in the pathogenesis of OFG although few and contrasting data are currently available on this issue. In a patient with OFG, we observed virtually complete CD4 and CD8 T-cell receptor (TCR) β-chain variable region (BV) repertoires at the lesion level and in circulation. However, oligoclonal profiles were found in CD4 and, to a greater extent, in CD8 subsets. These findings were seen in association with a massive peripheral T-cell activation, decreased naive T cells, reduced thymic output, altered cytokine production, and increased apoptosis. Our data, pointing to a random influx of T cells at the site of inflammation, argue against the hypothesis of a main allergen acting at the level of oral mucosa. The profound dysregulation of the peripheral T-cell compartment suggests that OFG should be regarded as a systemic disorder with localized manifestations.
正常人类受试者中 CD8 CD45RO 亚群中 T 细胞受体的克隆优势。
DOI: --
发表时间: 1993
期刊: Journal of immunology (Baltimore, Md. : 1950)
影响因子: --
作者:
Hingorani,R;Choi,IH;Akolkar,P;Gulwani-Akolkar,B;Pergolizzi,R;Silver,J;Gregersen,PK
通讯作者: Gregersen,PK