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。%IgG4 -related disease is a multi -system disease , which often manifests as elevated serum IgG 4 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 IgG 4-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 IgG 4-related kidney disease and idiopathic membranous nephropathy , in order to provide some theoretical foundations for clicinal diagnosis and treatment .
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