The diagnosis and treatment of the IgG4-related kidney disease

doi:10.3877/cma.j.issn.2095-3216.2013.01.006

Abstract

Abstract:

IgG4-related kidney disease is characterized by tubulointerstitial nephritis. Its diagnoses rely on a combination of clinical, serological, radiographic, and histopathologic features.Response to corticosteroids therapy is favorable. The sooner, the better therapy. So early diagnosis is very important. However, the pathogenesis is still unclear，although related to allergy or autoimmune function disorders. Therefor, here we try to discuss about the diagnostic criteria of both American Society of Nephrology and Japanese Society of Nephrology，in order to deepen the understanding of this disease.

Key words: IgG4-related kidney disease, Tubulointerstitial nephritis, Corticosteroids, Pathogenesis, Diagnostic criteria

Jing-di SUN, Wen-ge LI, Jiu-yang ZHAO. The diagnosis and treatment of the IgG4-related kidney disease[J]. Chinese Journal of Kidney Disease Investigation(Electronic Edition), 2013, 02(01): 28-33.

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References 36

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检查方法和部位	病变特点
组织学	富含浆细胞浸润的TIN，在细胞浸润最集中区域IgG4+浆细胞数＞10个/HP^a 免疫荧光或电镜检查毛细血管壁或TBM存在免疫复合物沉积^b
影像学	肾皮质小结节，圆形或楔形损害，或呈弥漫斑片状受累肾脏体积增大
实验室检查	血清IgG4或IgG水平升高高免疫球蛋白血症嗜酸粒细胞增多
其他受累器官	包括AIP、硬化性胆管炎、任何器官的炎性肿块、唾液腺和泪腺受累、炎性动脉瘤、肺部受累、腹膜后纤维化等

检查方法和部位	病变特点
组织学	富含浆细胞浸润的TIN，在细胞浸润最集中区域IgG4+浆细胞数＞10个/HP^a 免疫荧光或电镜检查毛细血管壁或TBM存在免疫复合物沉积^b
影像学	肾皮质小结节，圆形或楔形损害，或呈弥漫斑片状受累肾脏体积增大
实验室检查	血清IgG4或IgG水平升高高免疫球蛋白血症嗜酸粒细胞增多
其他受累器官	包括AIP、硬化性胆管炎、任何器官的炎性肿块、唾液腺和泪腺受累、炎性动脉瘤、肺部受累、腹膜后纤维化等

序号	病变特点
①	尿液、肾功能检查有任何异常，血清学检查有高IgG血症、低补体血症、高IgE血症中任何一个
②	影像学有特征性的异常改变（弥漫性肾脏肿大、肾实质多发性低密度区域、单发性肾肿瘤、肾盂壁肥厚）
③	有高IgG4血症（≥1350mg/L）
④	肾脏病理有以下两种改变： a.有明显的淋巴细胞、浆细胞浸润，以IgG4或IgG阳性浆细胞比≥40%、或IgG4阳性浆细胞数量＞10个/HP b.浸润细胞周围有特征性的纤维化改变
⑤	肾脏以外的脏器组织病理学检查有淋巴细胞、浆细胞浸润伴纤维化改变，以IgG4或IgG阳性浆细胞比≥40%、或IgG4阳性浆细胞数量＞10个/HP

序号	病变特点
①	尿液、肾功能检查有任何异常，血清学检查有高IgG血症、低补体血症、高IgE血症中任何一个
②	影像学有特征性的异常改变（弥漫性肾脏肿大、肾实质多发性低密度区域、单发性肾肿瘤、肾盂壁肥厚）
③	有高IgG4血症（≥1350mg/L）
④	肾脏病理有以下两种改变： a.有明显的淋巴细胞、浆细胞浸润，以IgG4或IgG阳性浆细胞比≥40%、或IgG4阳性浆细胞数量＞10个/HP b.浸润细胞周围有特征性的纤维化改变
⑤	肾脏以外的脏器组织病理学检查有淋巴细胞、浆细胞浸润伴纤维化改变，以IgG4或IgG阳性浆细胞比≥40%、或IgG4阳性浆细胞数量＞10个/HP

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