【摘要】 目的 探讨IgG4相关肾小管间质性肾炎(IgG4-TIN)临床病理学特征。方法 回顾性分析2例经病理学证实的IgG4-TIN病理学特点及免疫表型，并结合相关文献进行讨论。结果 2例均为中老年患者，血肌酐及IgG4水平均升高，肾小管轻-中度萎缩，肾间质内见大量淋巴细胞、浆细胞浸润，多灶及片状纤维化，局部呈席纹状或鸟眼样改变，小血管管壁增厚。免疫荧光IgG、IgG4局灶沉积于肾小管基底膜(TBM)。免疫组化提示肾间质浸润浆细胞弥漫表达CD138、CD38,且2例IgG4阳性浆细胞热点区>100个/HPF,IgG4阳性浆细胞/IgG阳性浆细胞>40%。其中病例1免疫荧光IgG、PLA2R显示2+,弥漫细颗粒状沉积于肾小球毛细血管襻，且电镜显示肾小球毛细血管襻上皮下、基底膜见大量电子致密物沉积，该例伴发PLA2R阳性膜性肾病。结论 IgG4-TIN少见，可并发膜性肾病等肾小球病变。依据其血清IgG4水平、典型病理学特点及免疫表型可作出正确诊断。糖皮质激素仍是其治疗首选药物。更多还原

【Abstract】 Objective To investigate the clinicopathological features of IgG4-related tubulointerstitial nephritis(IgG4-TIN). Methods The morphology and immunoprofiles of the IgG4-TIN were confirmed by pathology, and the relevant literatures were reviewed. Results Two cases were middle-aged and elderly patients, with elevated serum creatinine and IgG4 levels. H&E staining showed a large number of lymphocytes and plasma cells infiltration in the interstitium, multifocal and lamellar fibrosis with storiform and “bird’s eye” changes. The wall of small vessels was thickened. Immunofluorescence microscopy showed IgG and IgG4 deposited along the tubular basement membrane. Immunohistochemically, infiltrating plasma cells diffusely expressed CD138 and CD38. There were more than 100/HPF IgG4-positive plasma cells at the hot spots, and the ratio of IgG4-positive/IgG-positive plasma cells was over 40%. IgG and PLA2R(phospholipase A2 receptor)were concomitant positive along the glomerular capillary walls on immunofluorescence microscopy, and electron microscopy showed a large amount of electron dense deposits in the subepithelial and basement membrane of glomerular capillary loops. This case was associated with PLA2R-positive membranous nephropathy. Conclusion IgG4-TIN is a rare disease and may be complicated with membranous nephropathy and other glomerulopathy. It can be diagnosed according to the serum IgG4 level, typical histopathological characteristics and immunophenotype. Glucocorticoid is still the fist choice for treatment of IgG4-TIN.更多还原