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IgG4相关性疾病(IgG4-related disease,IgG4-RD)是一种免疫介导的纤维炎症性疾病,可致多器官受累并产生肿块、组织损伤和器官衰竭[1]。绝大多数IgG4-RD患者IgG4明显升高。当IgG4相关性疾病累及肾脏时即称为IgG4相关性肾病(IgG4-related kidney disease,IgG4-RKD)。IgG4相关性肾病的形式多种多样,最容易累及肾小管间质[2],此即为IgG4相关性小管间质性肾炎(IgG4-related tubulointerstitial nephr