Abstract: IgG4-related disease is a multi-system disease,which often manifests as elevated serum IgG4 concentrations,IgG4-positive plasma cell infiltration and fibrosis lesions.IgG4-related disease can affect many organs,such as pancreas,kidney,and salivary glands.When kidney is involved,we call it IgG4-related kidney disease,and its most common renal manifestation is IgG4-related tubulointerstitial nephritis.In addition,glomerulus involvement,most commonly membranous nephropathy（MN）,could also be found.Meanwhile,idiopathic membranous nephropathy is associated with high IgG4.It is important to distinguish between the MN secondary to IgG4-related disease and idiopathic membranous nephropathy,which is a separate disorder.In this paper,through the discussion from pathogenesis,clinical manifestation,diagnosis and treatment,we compared the differences between IgG4-related kidney disease and idiopathic membranous nephropathy，in order to provide some theoretical foundations for clicinal diagnosis and treatment.

Key words: Nephrosis, Immunoglobulin G, IgG4-related kidney disease, Idiopathic membranous nephropathy

摘要： IgG4相关性疾病是以血清IgG4升高、组织IgG4浆细胞浸润以及纤维性病变为特征的多系统疾病，可累及胰腺、肾脏、唾液腺等多种器官，累及肾脏时称为IgG4相关性肾病，主要表现为IgG4相关性肾小管间质性肾炎，累及肾小球时以膜性肾病多见。而特发性膜性肾病的免疫复合物沉积也以IgG4为主，在临床上区分IgG4相关性疾病继发的膜性肾病与特发性膜性肾病非常重要。本文从发病机制、临床表现、诊断及治疗等方面探讨IgG4相关性肾病与特发性膜性肾病的区别，为临床的诊治提供一定的理论基础。

关键词: 肾病, 免疫球蛋白G, IgG4相关性肾病, 特发性膜性肾病