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Pathogenesis of IgG4-related disease: a critical review

机译:IgG4相关疾病发病机制:批判性综述

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Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is a chronic, systemic, inflammatory condition of unknown etiology. Histopathologic examination is the key to diagnosis of IgG4-RD. The histopathologic features of IgG4-RD are lymphoplasmacytic infiltration, storiform fibrosis, and obliterative phlebitis. As for fewer than 15 years, IgG4-RD has been recognized as a unified diagnostic entity. CD4(+) T and B cells, which likely cause organ damage and disabling tissue fibrosis, constitute the major inflammatory cell population in patients with IgG4-RD. Affected patients with active, untreated disease have a marked expansion of IgG4-secreting plasmablasts in the blood. Important mechanistic insights regarding the pathogenesis of IgG4-RD have been gradually disclosed in recent years. Exploring the role of interactions between these CD4(+) T and B cells in patients with IgG4-RD is a highly promising field of investigation. In this review, we focus on CD4(+) T cell subsets and the T-cell clones that are involved in the pathogenesis of IgG4-RD.
机译:免疫球蛋白G4(IgG4) - 相关疾病(IgG4-RD)是一种慢性,全身性,未知病因的炎症状况。组织病理学检查是诊断IGG4-RD的关键。 IgG4-RD的组织病理学特征是淋巴基状渗透,储层纤维化和灭菌性静脉炎。少于15年,IgG4-RD已被认为是统一的诊断实体。可能导致器官损伤和致残组织纤维化的CD4(+)T和B细胞构成IgG4-RD患者的主要炎症细胞群。受影响的活性,未经处理的疾病的患者具有显着的IgG4分泌血浆中血液中的膨胀。近年来逐渐披露了关于IGG4-RD发病机制的重要机械洞察力。探索IGG4-RD患者中这些CD4(+)T和B细胞之间的相互作用的作用是一个高度有前途的调查领域。在本文中,我们专注于CD4(+)T细胞亚群和参与IgG4-RD发病机制的T细胞克隆。

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