IgG相关性疱病免疫球蛋白亚型分布的研究

发布时间：2018-01-21 08:02

本文关键词： IgG Ig G亚型补体临床病理学免疫荧光　出处：《福建医科大学》2015年硕士论文　论文类型：学位论文

【摘要】：[研究背景]Ig G相关性皮肤大疱性疾病是一组体内存在致病性抗自身Ig G组分的免疫性大疱性疾病,其中寻常型天疱疮和大疱性类天疱疮分别是天疱疮和类天疱疮最常见的类型。Ig G各亚型在疾病的发生、发展中起重要作用,检测其分布情况对疾病的诊断、治疗和预后评估具有重要意义。目前Ig G各亚型及补体在其中的相互作用机制尚不清楚,尚未见相关性文献。[目的]研究Ig G1、2、3和4亚型及补体C3d、C4d在寻常型天疱疮和大疱性类天疱疮中的表达,及其与组织形态学的相关性,探讨其在自身免疫性大疱性疾病发病中的作用。[方法]应用HE染色和直接免疫荧光(direct immunofluorescence,DIF)染色技术,分析大疱性疾病病理形态学特征以及Ig G、Ig G 1-4亚型、C3d、C4d免疫荧光染色情况。[结果]1.PV和BP相似的形态学表现:疱部位表皮的角化过度及角化不全、疱内炎症、真皮水肿、真皮淋巴细胞、中性粒细胞、嗜酸性粒细胞及少量浆细胞等炎症浸润等,并时有交叉。2.PV和BP差异的形态学表现:表皮内疱、水疱内棘层松解细胞、毛囊受累、墓碑及绒毛等在PV中的出现率高于BP,表皮下疱、齿缘样表现、疱顶变性坏死、嗜酸性粒细胞性海绵水肿、疱内炎症以及嗜酸性粒细胞浸润等在BP中出现率高于PV,BP水疱内炎症程度高于PV。3.PV和BP相似的免疫学表现:Ig G1-3亚型多伴有C3d和/或C4d的沉积,三分之一多的Ig G4+病例不伴其他Ig G亚型,Ig G各亚型的阳性率顺序、C3d和C4d阳性率基本一致。4.PV和BP差异的免疫学表现:Ig G各亚型及C3d和C4d的表达模式,PV呈表皮细胞间网格状沉积;BP为基底膜线性阳性。[结论]1.PV和BP均主要为Ig G4亚型,补体激活途径和非补体依赖性途径均参与PV和BP的发病。2.临床表现的重叠,组织形态学的交叉,免疫学表型的相似,我们提出PV和BP可能是同一种疾病的不同形态学谱系,需进一步行桥粒、半桥粒基因表型、SNPs、m RNA表达、micro RNA以及相关蛋白表达的检测得到确认。
[Abstract]:[Background] IGG-associated bullous skin disease is an immunological bullous disease with pathogenicity against autogenous Ig G in vivo. Among them, pemphigus vulgaris and bullous pemphigoid are the most common types of pemphigus and pemphigoid. Detection of its distribution is of great significance in the diagnosis, treatment and prognosis evaluation of the disease. At present, the mechanism of the interaction between the subtypes of IgG and complement in it is not clear, and no relevant literature has been found. [Objective] to study the expression of Ig G1O2M3 and 4 subtypes and complement C3dC4d in pemphigus vulgaris and pemphigoid bullous, and their correlation with histomorphology. To explore its role in the pathogenesis of autoimmune bullous disease. [Methods] HE staining and direct immunofluorescence staining were used. The pathomorphological features of bullous diseases and the immunofluorescence staining of C3dG1-4 subtype Ig G 1-4 were analyzed. [Results: 1. The morphological features of PV and BP were similar: keratosis and hypokeratosis of epidermis, inflammation, edema, lymphocytes and neutrophils in the epidermis of blister. Eosinophilic granulocytes and a small amount of plasma cells and other inflammatory infiltration, and sometimes cross-.2.PV and BP differences in morphology: epidermal blister, blisters in the spinous layer of loosening cells, hair follicle involvement. The occurrence rate of tombstone and villi in PV was higher than that in BP, subepidermal blister, edentulous appearance, epidermis degeneration and necrosis, eosinophil sponge edema. The incidence of intravesicular inflammation and eosinophil infiltration in BP was higher than that in PV. The degree of inflammation in BP vesicles was higher than that in PV.3.PV and BP. More than 1/3 cases of IgG4 without other IgG subtypes were found to be positive in the order of the positive rate of each subtype of IgG4. The positive rates of C3D and C4d were basically the same. 4. The immunological manifestations of the difference between PV and BP were as follows: 1. The expression patterns of C3d and C4d and the expression pattern of C3d and C4d were reticular deposition of epidermis cells. BP was linearly positive in basement membrane. [PV and BP are mainly Ig G4 subtypes. Complement activation pathway and complement independent pathway are involved in the pathogenesis of PV and BP. 2. The overlap of clinical manifestations and the cross of histomorphology. 2. We suggest that PV and BP may be different morphologic lineages of the same disease and need to be further expressed in desmosome and semi-desmosome gene phenotypes. The expression of micro RNA and related proteins was confirmed.
【学位授予单位】：福建医科大学
【学位级别】：硕士
【学位授予年份】：2015
【分类号】：R758.66

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