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论著:免疫球蛋白G4相关小管间质性肾炎2例报告及文献复习
Tubulointerstitial Nephritis Associated with IgG4-Related Systemic Disease: a Report of Two Cases and Literature Review
刘红 侯君 蔡锋晴 丁小强
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作者单位:复旦大学附属中山医院肾脏科
中文关键字:免疫球蛋白G4;小管间质炎症;自身免疫性疾病
英文关键字:IgG4; Tubulointerstitial nephritis; Autoimmune disease
中文摘要:目的:通过个案报告及文献复习,认识一种新的肾脏疾病——免疫球蛋白G4(IgG4)相关小管间质性肾炎。方法:分析2例IgG4相关小管间质性肾炎的诊断、病理表现及治疗经过,并复习相关文献。结果:IgG4相关小管间质肾炎是IgG4相关性系统性疾病累及肾脏时最常见的病变,患者除有高球蛋白血症、高IgG4血症、低补体血症外,肾脏损伤表现主要为少量蛋白尿、偶见镜下血尿伴肾功能损伤。肾间质中大量IgG4阳性浆细胞浸润、间质纤维化为其特点。糖皮质激素治疗有效。结论:IgG4相关性系统性疾病可以导致肾小管间质炎症,值得临床重视。
英文摘要:Objective: To study a new renal disease: IgG4 -related tubulointerstial nephritis. Methods: Analyze clinical feature, laboratory examination, renal biopsy, treatment and follow-up data of two case of tubulointerstitial nephritis associated with IgG4 -related systemic disease and review the literature. Results: IgG4-related systemic disease was a protean disorder that covers a wide variety of lesions. Tubulointerstitial nephritis was the major finding when kidney affected by it. The injuries were associated with a high level of serum IgG4 and abundant IgG4-positive plasma cell infiltration into the renal interstitial with fibrosis. Corticosteroids therapy is effective. Conclusions: We should pay more attention to IgG4-related autoimmune disease, which could be one cause of tubulointerstitial nephritis.
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