| 朱婷樱,姬婷婷,杜红秀,等.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[J].安徽医药,2024,28(10):2077-2082. |
| 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 IgG3 κdeposits: a case report |
| |
| DOI：10.3969/j.issn.1009-6469.2024.10.035 |
| 中文关键词: 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 |
| 英文关键词: Glomerulonephritis,membranoproliferative Monoclonal IgG3κ deposits Renal pathology Prednisone Cyclophosphamide Prognosis |
| 基金项目:上海自然科学基金项目( 21ZR1413900) |
|
| 摘要点击次数: 768 |
| 全文下载次数: 405 |
| 中文摘要: |
| 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 100 μmol/L下降至 71 μmol/L，24小时蛋白尿从 6 645 mg/24 h下降至 100 mg/24 h，尿白蛋白肌酐比从 4 882.3 mg/g下降至130.5 mg/g，尿蛋白肌酐比从 6 604.8 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。 |
| 英文摘要: |
| 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 QingpuBranch of Zhongshan Hospital affiliated to Fudan University from August 2021 to October 2022 were analyzed. Four years earlier, thepatient 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 ofrenal function. In August 2021, the patient was admitted to the Qingpu Branch of Zhongshan Hospital affiliated with Fudan Universitydue to significant proteinuria and edema. A repeated renal biopsy revealed membranoproliferative glomerulonephritis. Based on immunofluorescence and immunohistochemical results, PGNMID-IgG3κ was diagnosed, despite negative immunofixation electrophoresis andserum protein electrophoresis results. The treatment regimen included prednisone combined with cyclophosphamide (CTX) for fivemonths.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 100 μmol/L to 71 μmol/L, 24-hour proteinuria reduced from 6 645 mg/24 h to 100 mg/24 h, the urinary albumin-to-creatinine ratio decreased from 4 882.3 mg/g to 130.5 mg/g, and the urinary pro‐tein-to-creatinine ratio dropped from 6 604.8 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 resultsare negative. Early diagnosis and treatment are crucial for the prognosis of the disease. The combination of prednisone and CTX may bean effective treatment regimen for PGNMID-IgG3κ patients, improving their prognosis and extending survival. |
|
查看全文
查看/发表评论 下载PDF阅读器 |
| 关闭 |
