IgG4相关性疾病是一种新近认识的炎症性、纤维化性疾病,可累及单一或者多个器官,多见于中老年男性患者,儿童报道少见.肾脏损害统称为IgG4相关性肾脏疾病,可表现为IgG4相关性肾小管间质性肾炎、膜性肾病和肾盂病变等,该疾病的诊治在国内外逐步受到重视,为了提高对该疾病的认识,该文综述了其临床表现、实验室检查特点、病理特征和诊治进展.%IgG4-related disease is a newly recognized systematic inflammatory and fibrotic disease which affects one or more organs.This disease mainly occurs in the middle-aged and old males.Currently only a few cases were reported in children.Renal involvement was referred to as IgG4-related kidney disease,including IgG4-related tubulointerstitiai nephrisis,membraneous nephropathy and renal pyelitis.The clinical features,laboratory tests,pathological findings and treatment progresses have been summarized in this review.
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