目的： 分析C₃肾小球病（C₃ glomerulopathy，C₃G）的临床预后相关病理特征。方法： 收集2012年至2022年于上海交通大学医学院附属瑞金医院肾内科住院且经肾活检组织学诊断为C₃G的所有病例，共7例，回顾性分析其临床、病理及随访资料，观察预后相关病理特征。结果： 7例C₃G患者（男/女，3/4），中位起病年龄51岁，中位确诊年龄为54岁，中位随访时间为84个月（48~144个月），2例失随访，2例预后不良，3例预后良好。2例患者临床表现为肾病综合征，3例伴肾功能不全，6例伴有血尿，7例均表现为低补体血症伴高血压，且均无明显肾外表现。长期随访患者5例，随访至2年时，1例尿蛋白及肌酐完全缓解，3例尿蛋白及肾功能稳定，1例尿蛋白增加、肾功能下降；随访至6年时3例预后较好，1例在随访2年时预后差的基础上尿蛋白进一步增加，肾功能明显下降，1例新进展至大量尿蛋白。随访6年时，回顾肾脏病理，3例预后好的病例以弥漫毛细血管内皮细胞增生伴中性粒细胞浸润为主要表现，而肾小球球性硬化及毛细血管襻假双轨表现不明显，且系膜区电子致密物沉积较少；2例预后差的病例以肾小球球性硬化比例升高、大量毛细血管襻假双轨、系膜区大量电子致密物沉积为主要表现，而毛细血管内皮细胞增生伴中性粒细胞及嗜酸性粒细胞浸润较少。结论： C₃G组织病理以急性病变（肾小球毛细血管内皮细胞增生、中性粒细胞浸润等）为主要表现者，预后较好，可予以激素、免疫抑制剂为主的积极治疗；C₃G病理组织表现以慢性病变（肾小球球性硬化、毛细血管襻假双轨形成、系膜区电子致密物沉积量等）为主要表现，患者则预后不良，建议予以保守治疗，避免过度治疗。

Objective To analyze pathological features related to clinical prognosis in C₃ glomerulopathy. Methods A total of seven cases diagnosed as C₃ nephropathy by renal biopsy in the Department of Nephrology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine from 2012 to 2022 were collected. The clinical, pathological and follow-up data were retrospectively analyzed, and the prognostic pathological features were observed. Results Among the 7 patients (male/female, 3/4), the median onset age was 51 years, the median age at diagnosis was 54 years, and the median follow-up time was 84 months (ranging from 48 to 144 months). Two patients were lost to follow-up, 2 patients showed poor prognosis, and 3 patients had good prognosis. Clinically, 2 cases presented with nephrotic syndrome, 3 cases had renal insufficiency, and 6 cases had hematuria. All 7 patients exhibited hypocomplementemia and hypertension, with no obvious extrarenal manifestations. Among the 5 patients with long-term follow-up, by the 2-year follow-up, 1 patient had complete remission of proteinuria and creatinine levels, 3 patients had stable proteinuria and renal function, and 1 patient had increased proteinuria and declining renal function. By the 6-year follow-up, 3 patients had good prognosis, 1 patient who had poor prognosis at the 2-year follow-up showed further increase in proteinuria and significant decline in renal function, and 1 patient progressed to massive proteinuria. At the 6-year follow-up, a retrospective analysis of renal pathology revealed that 3 cases with good prognosis primarily exhibited diffuse endothelial cell proliferation with neutrophil infiltration, with minimal glomerular sclerosis and faint double-track appearance of the capillary loops. In these cases, there was also limited deposition of electron-dense material in the mesangial area.. Two cases with poor prognosis showed an increased proportion of glomerular sclerosis, extensive double-track appearance of capillary loops, and significant deposition of electron-dense material in the mesangial area, while endothelial cell proliferation with neutrophil and eosinophil infiltration was less pronounced. Conclusions Acute pathological changes (such as glomerular capillary endothelial cell proliferation and neutrophil infiltration) are the main manifestations of C₃ nephropathy, which is associated with a good prognosis and can be treated aggressively with steroids and immunosuppressants. The pathological manifestations of C₃ are mainly chronic lesions (including glomerular sclerosis, double-track formation of capillary loops, and extensive deposition of electron-dense material in the mesangial area). C₃ patients have poor prognosis, and conservative treatment is recommended to avoid excessive treatment.