强的松联合环磷酰胺治疗单克隆IgG3κ沉积的增生性肾小球肾炎1例  

Prednisone combined with cyclophosphamide for proliferative glomerulonephritis with monoclonal IgG3κdeposits:a case report

作　　者：朱婷樱 姬婷婷 杜红秀 查芳芳 朱迎春 熊晓燕 白寿军 ZHU Tingying;JI Tingting;DU Hongxiu;ZHA Fangfang;ZHU Yingchun;XIONG Xiaoyan;BAI Shoujun(Department of Nephrology,Qingpu Branch of Zhongshan Hospital,Fudan University,Shanghai 201700,China)

机构地区：[1]复旦大学附属中山医院青浦分院肾内科,上海201700

出　　处：《安徽医药》2024年第10期2077-2082,I0007,共7页Anhui Medical and Pharmaceutical Journal

基　　金：上海自然科学基金项目(21ZR1413900)。

摘　　要：目的研究单克隆免疫球蛋白G沉积的增生性肾小球肾炎(PGNMID)的早期诊断及治疗。方法分析复旦大学附属中山医院青浦分院1例66岁单克隆免疫球蛋白G3κ沉积的增生性肾小球肾炎女性从2021年8月至2022年10月的临床资料,病人4年前因尿中泡沫增多就诊行肾活检提示膜增生性肾小球肾炎。既往长期口服黄芪颗粒及血管紧张素受体拮抗剂(ARBs)保守治疗,随访肾功能缓慢进展。2021年8月病人因大量蛋白尿及水肿就诊于复旦大学附属中山医院青浦分院,重复肾穿刺,肾脏病理检查提示膜增生性肾小球肾炎,结合免疫荧光及免疫组化结果,考虑单克隆免疫球蛋白G3κ(IgG3κ)沉积的增生性肾小球肾炎,免疫固定电泳及血清蛋白电泳阴性,故诊断为PGNMID-IgG3κ型。治疗方案选择强的松联合环磷酰胺(CTX),治疗5个月。结果病人疾病完全缓解,随访14个月疾病无复发。血白蛋白从治疗前的21 g/L升高至41 g/L,血肌酐从100μmol/L下降至71μmol/L,24小时蛋白尿从6645 mg/24 h下降至100 mg/24 h,尿白蛋白肌酐比从4882.3 mg/g下降至130.5 mg/g,尿蛋白肌酐比从6604.8 mg/gcr至242.6 mg/gcr。结论PGNMID发病机制目前仍未完全了解。当怀疑该病时,即使免疫固定电泳等阴性,也需行IgG亚型及轻链染色检查。早期诊断及治疗对疾病的预后极为重要;强的松联合CTX治疗可能是PGNMID-IgG3κ病人一种较好的治疗方案,能够改善病人预后,延长其生存期。Objective To investigate the early diagnosis and treatment of proliferative glomerulonephritis with monoclonal immunoglobulin G deposits(PGNMID).Methods The clinical data of a 66-year-old female patient with PGNMID admitted to the Qingpu Branch of Zhongshan Hospital affiliated to Fudan University from August 2021 to October 2022 were analyzed.Four years earlier,the patient presented with increased urinary foam and was diagnosed with membranoproliferative glomerulonephritis based on a renal biopsy.She had been conservatively treated with Astragalus granules and angiotensin receptor blockers(ARBs),with gradual progression of renal function.In August 2021,the patient was admitted to the Qingpu Branch of Zhongshan Hospital affiliated with Fudan University due to significant proteinuria and edema.A repeated renal biopsy revealed membranoproliferative glomerulonephritis.Based on immunofluorescence and immunohistochemical results,PGNMID-IgG3κwas diagnosed,despite negative immunofixation electrophoresis and serum protein electrophoresis results.The treatment regimen included prednisone combined with cyclophosphamide(CTX)for five months.Results The patient achieved complete remission,with no recurrence observed during a 14-month follow-up.Serum albumin levels increased from 21 g/L to 41 g/L,serum creatinine decreased from 100μmol/L to 71μmol/L,24-hour proteinuria reduced from 6645 mg/24 h to 100 mg/24 h,the urinary albumin-to-creatinine ratio decreased from 4882.3 mg/g to 130.5 mg/g,and the urinary protein-to-creatinine ratio dropped from 6604.8 mg/gcr to 242.6 mg/gcr.Conclusions The pathogenesis of PGNMID remains unclear.When PGNMID is suspected,IgG subtype and light chain staining should be performed even if immunofixation electrophoresis results are negative.Early diagnosis and treatment are crucial for the prognosis of the disease.The combination of prednisone and CTX may be an effective treatment regimen for PGNMID-IgG3κpatients,improving their prognosis and extending survival.

关 键 词：肾小球肾炎 膜增生性 单克隆IgG3κ沉积 肾脏病理 强的松 环磷酰胺 预后 

分 类 号：R692.31[医药卫生—泌尿科学]