诊断学理论与实践

诊断学理论与实践 ›› 2024, Vol. 23 ›› Issue (06): 587-593.doi: 10.16150/j.1671-2870.2024.06.005

• 论著 • 上一篇    下一篇

C3肾病临床预后相关病理特征分析

张俊花1,2, 李一林3, 谢静远2, 张春丽2, 徐静2()   

  1. 1.山西省第二人民医院肾内科，山西 太原 030012
    2.上海交通大学医学院附属瑞金医院肾脏科，上海 200025
    3.江苏省苏州市中医医院，江苏 苏州 215002
  • 收稿日期:2024-06-27 出版日期:2024-12-25 发布日期:2024-12-25
  • 通讯作者: 徐静 E-mail：xjjesse@126.com
  • 基金资助:
    国家重点基础研究发展计划（973计划）(2012CB517600(2012CB517606))

Analysis of pathological features related to clinical prognosis in C3 glomerulopathy

ZHANG Junhua1,2, LI Yilin3, XIE Jingyuan2, ZHANG Chunli2, XU Jing2()   

  1. 1. Department of Nephrology, Second People’s Hospital of Shanxi Province, Taiyuan 030012, China
    2. Department of Nephrology, Ruijin Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Shanghai 200025, China
    3. Suzhou Hospital of Traditional Chinese Medicine, Suzhou 215002, China
  • Received:2024-06-27 Published:2024-12-25 Online:2024-12-25

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摘要：

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（例1、例7）失随访，2例（例5、例6）预后不良，3例（例2~4）预后良好。临床表现，2例（例3、例7）为肾病综合征，3例（例1、例3、例6）伴肾功能不全，6例（例1~6）伴有血尿，7例均表现为低补体血症伴高血压，均无明显肾外表现。长期随访患者5例，随访至2年时，1例（例3）尿蛋白及肌酐完全缓解，3例（例2、例4、例5）尿蛋白及肾功能稳定，1例（例6）尿蛋白增加、肾功能下降。随访至6年时，2例预后较好（例2、例4），1例（例3）因尿蛋白及肌酐完全缓解停止随访，1例（例6）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（例5）新进展至大量尿蛋白。随访6年时，回顾肾脏病理，3例（例2~4）预后好的病例，光镜下以弥漫毛细血管内皮细胞增生伴毛细血管管腔内中性粒细胞浸润为主要表现，而肾小球球性硬化及肾小球基底膜假双轨表现不明显，且电镜下系膜区电子致密物沉积较少；2例（例5、例6）预后差的病例，以光镜下肾小球球性硬化比例升高[大于（年龄-20）/2×100%]、大量肾小球基底膜假双轨，电镜下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毛细血管腔内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、管腔中性粒细胞浸润等）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组织病理表现以慢性病变（ 肾小球球性硬化、肾小球基底膜假双轨形成、系膜区电子致密物大量沉积等）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。

关键词: C3肾病, 临床表现, 肾脏病理, 预后

Abstract:

Objective To analyze the pathological features associated with the clinical prognosis of C3 glomerulopathy (C3G). Methods A total of consecutive 7 cases, all hospitalized in the Department of Nephrology, Ruijin Hospital Affiliated to Shanghai Jiao Tong University School of Medicine between 2012 and 2022, and histologically diagnosed with C3G by renal biopsy, were collected. Their clinical, pathological, and follow-up data were retrospectively analyzed to observe pathological characteristics related to prognosis. Results The cohort consisted of 7 C3G patients (male/female: 3/4). The median age at onset was 51 years, and the median age at diagnosis was 54 years. The median follow-up time was 84 months (range: 48-144 months). Two patients (Case 1, Case 7) were lost to follow-up. Two patients (Case 5, Case 6) had a poor prognosis, while three patients (Case 2-4) had a favorable prognosis. Clinically, 2 patients (Case 3, Case 7) presented with nephrotic syndrome, 3 patients (Case 1, Case 3, Case 6) had renal insufficiency, 6 patients (Case 1-6) had hematuria, and all 7 patients exhibited hypocomplementemia and hypertension, without significant extrarenal manifestations.Among the 5 patients with long-term follow-up, at the 2-year mark: 1 patient (Case 3) achieved complete remission of proteinuria and serum creatinine; 3 patients (Case 2, Case 4, Case 5) had stable proteinuria and renal function; and 1 patient (Case 6) showed increased proteinuria and declining renal function. At the 6-year follow-up: 2 patients (Case 2, Case 4) maintained a good prognosis; 1 patient (Case 3) was no longer followed up after achieving complete remission of proteinuria and creatinine; 1 patient (Case 6), who already had a poor prognosis at 2 years, exhibited a further increase in proteinuria and a significant decline in renal function; and 1 patient (Case 5) newly progressed to heavy proteinuria.At the 6-year follow-up, a review of renal pathology revealed that the 3 cases with a favorable prognosis (Case 2-4) primarily showed diffuse capillary endothelial cell hyperplasia with neutrophil infiltration on light microscopy, while global glomerulosclerosis and glomerular basement membrane pseudo-double contour formation were not prominent. Electron microscopy demonstrated relatively few electron-dense deposits in the mesangial region. In contrast, the 2 cases with a poor prognosis (Case 5, Case 6) were characterized by an increased proportion of global glomerulosclerosis[ greater than (age-20)/2 × 100%] ,extensive glomerular basement membrane pseudo-double contours on light microscopy, and abundant mesangial electron-dense deposits on electron microscopy, with minimal capillary endothelial cell hyperplasia and neutrophil infiltration. Conclusions C3G patients whose histopathology is predominantly characterized by acute lesions (such as glomerular capillary endothelial cell hyperplasia and neutrophil infiltration) have a more favorable prognosis and can be treated aggressively with corticosteroids and immunosuppressants. Conversely, C3G patients whose histopathology is predominantly characterized by chronic lesions (such as global glomerulosclerosis, glomerular basement membrane pseudo-double contour formation, and a high quantity of mesangial electron-dense deposits) have a poor prognosis, and conservative management is recommended to avoid overtreatment.

Key words: C3 glomerulopathy, Clinical manifestation, Renal pathology, Prognosis

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