IgG4-related disease (IgG4-RD) is an immune-mediated fibroinflammatory disorder that can affect multiple organs throughout the body, predominantly in middle-aged and elderly males, with a male-to-female ratio of 2꞉1 to 3꞉1. IgG4-related retroperitoneal fibrosis (IgG4-RPF), a rare subtype of IgG4-RD, has an unclear etiology, and its comorbidity with type 2 diabetes mellitus is also uncommon. A lack of awareness of this condition in clinical practice can easily lead to misdiagnosis. On July 14, 2016, the Third Xiangya Hospital of Central South University admitted a patient with type 2 diabetes mellitus complicated by IgG4-RPF. Following comprehensive treatment, including blood glucose and blood pressure control, kidney protection, circulation improvement, and the use of prednisone, the patient's condition significantly improved. The retroperitoneal fibrotic mass decreased in size, renal function improved, and serum IgG4 levels decreased. After 8 years of follow-up, the condition did not recur. Analyzing this case in conjunction with a literature review suggests that the development of IgG4-RPF in diabetic patients may be related to chronic inflammation from metabolic syndrome and atherosclerotic plaques associated with long-standing diabetes. This provides valuable clinical ideas for clinicians in diagnosing and treating this rare comorbidity.
IgG4相关性疾病(IgG4-related disease，IgG4-RD)是一种由免疫介导的纤维炎性反应性疾病，可以累及全身多器官，且好发于中老年男性，男女比例2꞉1~3꞉1。IgG4相关性腹膜后纤维化(IgG4-related retroperitoneal fibrosis，IgG4-RPF)作为IgG4-RD中的一种罕见类型，病因尚不明确，其与2型糖尿病的共病现象也较为罕见。临床上对该病的认识不足，易造成误诊。中南大学湘雅三医院于2016年7月14日收治1例2型糖尿病并发IgG4-RPF的患者，经降糖、降压、护肾、改善循环、应用激素等综合治疗后，病情较前明显好转，腹膜后纤维化包块较前明显缩小，肾功能好转，IgG4水平下降，且随访8年未再复发。对该病例进行深入分析并结合文献复习发现糖尿病合并IgG4-RPF的发生可能与代谢综合征产生的慢性炎症和长病程糖尿病的动脉粥样硬化斑块有关，这为临床医生提供了诊治思路。.
Keywords: IgG4; bilateral hydronephrosis; clinical diagnosis and treatment; retroperitoneal fibrosis; type 2 diabetes mellitus; ureteral stenosis.