以毛细血管内皮细胞弥漫增生为主要表现的IgA肾病的临床病理特点及其预后

目的探讨以毛细血管内皮细胞弥漫性增生为主要表现的IgA肾病(EPIgAN)的临床、病理特点和预后。方法分析北京大学第一医院近15年来IgA肾病(IgAN)的临床和病理资料,比较EPIgAN与非EPIgAN临床病理特点和肾脏存活率;分析EPIgAN预后及其影响因素;观察激素治疗对EPIgAN预后的影响。结果920例IgAN中符合EPIgAN47例,占5·1%。EPIgAN与非EPIgAN相比,肾穿时尿蛋白升高、高血压和水肿多见,细胞新月体明显,而肾小球硬化和肾间质纤维化则较轻;对所有研究对象进行随访,其中36例EPIgAN患者完成随访,平均随访62个月。100例非EPIgAN患者完成随访,平均随访时间64个月。两组共7例到达随访终点。Kaplan-Meier分析两组自然预后差异无显著性(LogRank,P=0·52);Cox回归分析内皮弥漫增生不是影响IgAN预后的危险因素(P=0·27);激素治疗能降低EPIgAN尿蛋白,但随访期内肾脏存活率与对照组差异无显著性。结论EPIgAN肾穿时临床表现重、组织活动性病变多而慢性化指标少,内皮弥漫增生不是影响IgAN预后的危险因素。

Clinical and pathological characteristics and prognosis of IgA nephropathy with diffused endocapillary proliferation

Objective To investigate the clinical and pathological characteristics and the prognosis of IgA nephropathy with diffused endocapillary proliferation(EPIgAN).Methods To analyse the clinical and pathological data of IgAN patients of Peking University First Hospital during the recent 15 years,and the clinical,pathological and renal survival rate of EPIgAN were compared with that of non-EPIgAN.The prognosis of EPIgAN and its risk factors were analyzed and the effectiveness of corticosteroids was also investigated .Results A total of 920 IgAN patients were included in this study.EPIgAN accounted for 5.1%.Hypertention,heavy proteinuria and edema were more popular in EPIgAN patients than in non-EPIgAN.Cellular crescents was more obvious in EPIgAN,while glomeruler sclerosis and renal interstitial fibrosis was less common.There was no difference between two groups in natural prognosis(Log rank,p=0.52).Diffused endocapillary proliferation was not a risk factor of the IgAN(OR=1.67,95%CI,1.70~4.11,P=0.27).Steroid therapy could reduce proteinuria of EPIgAN,but 5-year renal survival rate was not higher than non-steroid therapy patients.Conclusion Compared to non-EPIgAN patients,EPIgAN patients have more prominent clinical manifestations at biopsy,more active lesions and less chronic lesions in renal histology.Diffused endocapillary proliferation is not a risk factor of IgAN.