IgG4相关性疾病是一类原因不明的慢性、进行性炎症伴纤维化和硬化的罕见疾病,其主要特征为血清IgG4(血清免疫球蛋白的一种,参与人体免疫反应)水平显著增高,可累及唾液腺、胰腺、胆管、腹膜、肺脏、肾脏、垂体等多个器官,受累器官组织由于大量淋巴细胞和IgG4阳性浆细胞浸润伴纤维化,而发生肿大或结节、硬化性病变。 IgG4...
因此,IgG4相关性病变(IgG4-related disease,IgG4-RD)作为一个全新的自身免疫性疾病实体越来受到重视。已有报道称IgG4可累及脑膜,引发IgG4相关肥厚性硬脑膜炎(IgG4-related hypertrophic pachymeningitis,IgG4-RHP) [ 3 , 4 , 5 , 6 ] 。对部分原诊断为特发性肥厚性硬脑膜炎的患者,进行病理切片观察及IgG...
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...
目的探討IgG4相關性疾病(IgG4-related disease, IgG4-RD)的臨床病理特點,免疫表型,加強臨床和病理醫生對本病的認識。 方法分析15例IgG4-RD的臨床特點及病理改變,並複習相關文獻。 結果15例IgG4-RD平均年齡66歲,男女比7 : 3。發病部位分别為頜下腺(6例),腮腺(2例),淋巴結(5例),肺(1例)、睾丸(1例)。病...
IgG4-related disease. N Engl J Med. 2012 Feb 9;366(6):539-51. doi: 10.1056/NEJMra1104650. Review JH Stone,JK Chan,V Deshpande,... - 《International Journal of Rheumatology》 被引量: 245发表: 2015年 Mechanisms of disease: IgG4-related disease 539-551. http://dx.doi.org/10.1056/...
IgG4相关性疾病(IgG4-related disease,IgG4-RD)被认为是一种新的由免疫介导的自身免疫性疾病 [ 1 , 2 ]。该类疾病与IgG4淋巴细胞密切相关,以血清IgG4水平升高以及IgG4阳性浆细胞浸润多种器官和组织,伴有组织纤维化而发生肿大或结节性/增生性病变为特征。最常见的受累器官包括胰腺和唾液腺,其次可以发生于胆管、...
S. Nakamura, T. Ito, K. Notohara, T. Sumida, Y. Tanaka, T. Mimori, T. Chiba, M. Mishima, T. Hibi, H. Tsubouchi, K. Inui, H. Ohara, Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD),...
Zen, V. Deshpande IgG4-related disease. N. Engl. J. Med., 366 (6) (2012), pp. 539-551 10.1056/NEJMra1104650 22316447 View in ScopusGoogle Scholar 2. Y. Zen, Y. Nakanuma Pathogenesis of IgG4-related disease. Curr. Opin. Rheumatol., 23 (1) (2011), pp. 114-118 10.1097/BOR.0...
doi:10.1056/nejmc1614275Faiz KarimMarian Clahsen-van GroningenJan A.M. van LaarMassachusetts Medical SocietyNew England Journal of MedicineKarim F, Clahsen-van Groningen M, van Laar JA (2017) AA amyloidosis and IgG4-related disease. N Engl J Med 376(6):599-600...
An immunoglobulin G4 (IgG4)-related disease is important disease in differential diagnosis of tumors in kidney, pancreas, lung and other organs. The imaging findings of IgG4-related kidney diseases are usually expressed as defect contrast region, while c