IgG4-related disease is a newly described systemic fibroinflammatory process, characterized by increase in IgG4-positive plasma cells. Its pathogenesis, including the role of IgG4, remains poorly understood. Pl
近10年研究发现IgG4在IgG4自身免疫性疾病(IgG4 autoimmune diseases ,IgG4-AIDs)和IgG4相关性疾病(IgG4-related diseases,IgG4-RDs)中有致病作用。IgG4-AIDs是新出现的一组自身免疫病,其主要特点是出现IgG4 型自身抗体[2]. IgG4-AID自身抗原存在于在身体各个部位,主...
IgG4-related disease (IgG4-RD) is a systemic, immune-mediated fibroinflammatory condition capable of affecting multiple organs.IgG4相关疾病是一种系统性、免疫介导的纤维炎症性疾病,可累及多个器官。 Key Features (主要特点): Pathology (病理特点):Lymphoplasmacytic infiltrates, storiform fibrosis, and ...
inflammatory aortic aneurysm retroperitoneal fibrosis (Ormond disease) Riedel thyroiditis sclerosing mesenteritis Also called IgG4-associated disease IgG4-related systemic disease IgG4-related sclerosing disease IgG4-related systemic sclerosing disease
(IgG4-related disease,IgG4-RD);唾液腺(salivary gland);Mikulicz病(Mikulicz disease);Küttner瘤(Küttner tumor);腮腺(parotid gland);下颌下腺(submandibular gland);泪腺(lacrimal gland);眼眶(orbital);鼻旁窦(paranasal sinuses);鼻腔...
The concept of IgG4-related disease (IgG4-RD) as a systemic fibroinflammatory disease has been introduced in the very recent past. Many formerly thought "single-organ diseases" are now being summarized as organ manifestations of IgG4-RD. Therefore, much of the literature focusing on the ...
Immunoglobulin G4-related disease (IgG4-RD) is a systemic inflammatory disease characterized by chronic activation of the immune system with subsequent tissue fibrosis. It can affect a wide variety of different organ systems. The most common form of renal involvement (IgG4-RKD) is tubulointerstitial...
IgG4相关性疾病(IgG4-related disease,IgG4-RD)是一种新近认识的炎症性疾病,IgG4 +浆细胞浸润、受累器官呈席纹状纤维化和闭塞性静脉炎是该疾病显著的临床特征,可累及肾脏、胰腺、肝脏、唾液腺、泪腺、主动脉周区域和胆管等全身多个脏器系统 [ 1 , 2 ]。对肾脏的损害涵盖肾小管间质、肾小球和肾盂等,表现为...
IgG4相关眼病(immunoglobulin-G4 related ophthalmic disease,IgG4-ROD)是一种眼部由免疫介导的慢性炎症伴纤维化性的疾病,主要组织病理表现为IgG4+浆细胞浸润,并伴有席纹状纤维化、闭塞性静脉炎和嗜酸性粒细胞浸润。显著升高的血清IgG4水平和肿块样病变是该...
IgG4-related disease (IgG4-RD) represents an immune-mediated fibroinflammatory condition with peculiar histopathologic changes that can affect various organs. In 2012 its unified nomenclature was published, which allows to abandon other synonymous names. Up to now, only little is known about its epi...