目的 分析增生性肾小球肾炎伴单克隆免疫球蛋白沉积（PGNMID）患者的临床病理特点、治疗和预后。 方法 回顾性分析经肾活检诊断为PGNMID患者的临床资料、肾脏病理、治疗及预后情况。根据肾脏缓解情况，分为缓解组和未缓解组。对比两组之间人口统计学、实验室数据。 结果 共纳入28例患者，分为缓解组（n = 16）和未缓解组（n = 12），男女比例4：3，临床表现为蛋白尿（100%）、血尿（82.1%）、肾功能不全（28.6%）、低补体C3和（或）C4血症（32.1%）。1例（3.6%）患者检测到血清单克隆免疫球蛋白，5例（17.9%）患者血清游离轻链比值异常。肾脏组织病理学免疫荧光以IgG3沉积为主的共24例，以IgG3κ型最多见（17例），IgG1共4例。光镜下，有24例（85.7%）表现为MPGN，3例（10.7%）表现为EPGN，1例（3.6%）表现为MGN；电镜下可见电子致密物大多沉积在系膜区、内皮下，少数伴上皮下沉积。随访时间3 ~ 56个月，截至末次随访，共有5例患者进入ESRD，其中2例患者因心血管事件死亡，1例行腹膜透析，1例行血液透析，1例行肾脏替代治疗前准备。随访1、2、3年肾脏总缓解率分别为32.1%、57.1%、64.2%。缓解组的年龄（44 ± 17.35）岁，男8例（50%），女8例（50%）；未缓解组的年龄（59.83 ± 18.09）岁，男8例（66.7%），女4例（33.3%）；未缓解组的年龄、nT-proBNP、肌钙蛋白I、尿素氮均高于缓解组，差异有统计学意义（P < 0.05）；肾脏完全缓解的5例患者中，2例治疗方案为BD（硼替佐米联合地塞米松），1例为Dara单抗，其余2例为激素联合免疫抑制剂。 结论 PGNMID患者临床常表现为蛋白尿、血尿、肾功能不全；光镜下主要表现为MPGN，IgG3κ是最常见的亚型。基于硼替佐米的治疗可获得较好的血液学及肾脏缓解率，Dara单抗可能是治疗PGNMID安全且有效的药物，最佳治疗方案有待进一步探索。

Objective To analyze the clinicopathological features， treatment and prognosis of patients with proliferative glomerulonephritis with monoclonal immunoglobulin deposition. Methods The clinical data， renal pathology， treatment and prognosis of patients with PGNMID diagnosed by renal biopsy in Xijing Hospital from January 2018 to January 2024 were retrospectively analyzed and summarized. Results A total of 28 patients were enrolled in this study， with a male to female ratio of 4：3. The clinical manifestations were proteinuria （100%）， hematuria （82.1%）， renal insufficiency （28.6%）， and low complement C3 or C4 emia （32.1%）. One patient （3.6%） had serum monoclonal immunoglobulin， and 5 patients （17.9%） had abnormal serum free light chain ratio. The deposition of immunoglobulin in the kidney was IgG3 in 24 cases， IgG1 in 4 cases， and IgG3κwas the most common type （17 cases）. Then there are 24 cases （85.7%） of MPGN， 3 cases （10.7%） characterized by EPGN， 1 case （3.6%） of MGN. Under electron microscope， most of the electron-dense deposits were found in the mesangial area and subendothelium， and a few were accompanied by subepithelial deposits.The follow-up time ranged from 3 to 56 months. Until the last follow-up， a total of 5 patients entered ESRD， of which 2 patients died， 1 patient underwent peritoneal dialysis， 1 patient underwent hemodialysis， and 1 patient underwent preparation for renal replacement therapy. Follow-up of 1 year， 2 years， 3 years renal total response rate of 32.1%， 57.1% and 64.2% respectively. According to the situation of kidney ease into remission group （n = 16） and non-remission group （n = 12）. The average age of the remission group was 44 ± 17.35 years，including 8 males （50%）； The average age of the non-remission group was 59.83 ± 18.09 years， including 8 males （66.7%）. The age， nT-proBNP， troponin I and urea nitrogen of the non-remission group were higher than those of the remission group， and the differences were statistically significant （P < 0.05）. Among the 5 patients with renal complete remission， 2 were treated with BD （bortezomib combined with dexamethasone）， 1 was treated with Dara， and the remaining 2 were treated with steroids combined with immunosuppressants. Conclusions The clinical manifestations of PGNMID are proteinuria， hematuria and renal insufficiency. Under light microscope， MPGN was the main manifestation， and IgG3κwas the most common subtype. Based on bortezomib treatment can obtain good hematology and kidney remission rate.Dara may be a safe and effective drug for the treatment of PGNMID， the best treatment needs further exploration.