摘要
目的:了解单克隆IgG沉积的增生性肾小球肾炎临床病理特点。方法:回顾性分析单克隆IgG沉积的增生性肾小球肾炎患者的临床病理资料。结果:9例患者中男性5例,女性4例,肾活检时平均年龄49.8±10.9岁,肾脏病病程15.9±13.1月;血清白蛋白29.0±5.5g/L,8例患者血清白蛋白降低,尿蛋白定量5.6±2.8g/d,7例伴镜下血尿,尿N-乙酰-β-D-氨基葡萄糖苷酶72.3±38.6U/(g·cr),尿视黄醇结合蛋白11.3±17.1mg/L,血清肌酐(SCr)209.5±176.8μmol/L,7例患者SCr升高。补体C3下降5例,补体C4均正常。高血压8例,贫血8例,浆细胞均未见异常,7例行血清免疫固定电泳仅1例存在血清异常IgGκ型条带。肾活检病理光镜为膜增生样病变,3例亦见肾小球结节,1例伴新月体。电镜下见肾小球基膜内皮下、系膜区及少量上皮侧颗粒状电子致密物,其中2例电子致密物呈晶格状结构。患者均有IgG和C3在肾小球内的沉积,5例伴C1q沉积。IgG3κ型3例,IgG3λ型3例,IgG1κ型2例,IgG1λ型1例。结论:单克隆IgG沉积的增生性肾小球肾炎以中老年多见,临床表现大量蛋白尿、血尿,多数患者肾功能不全和贫血,部分出现血清异常单克隆条带。组织学为肾小球膜增生样病变,肾小球见颗粒状电子致密物,少数呈晶格状结构,沉积物以IgG3亚型多见。
Objective： To explore the clinical and pathological features of proliferative glomerulonephritis with monoclonal IgG deposits （PGNMID） from Chinese patients. Methodology： From Jun 2010 to Aug 2011, nine patients were diagnosed as PGNMID. Their clinical and pathological features were retrospectively analyzed. The diagnosed criteria of PGNMID was proliferative or membranoproliferative glomerulonephritis on LM, monoclonal IgG and staining for a single light-chain isotype on IF, and glomerular electron-dense deposits on EM. Results： Patients were 5 males and 4 females with an average age of 49.8±10.9 years old （ranged from 31～69）. The duration of renal disease was 15.9±13.1 months（1～36 months）. All of them had proteinuria with an average 5.60？2.80 g/24h of urine protein excretion （ranged from 2.56~10.1）, 7 had microscopic hematuria and 8 had hypoalbuminia with an average serum albumin of 29.0？5.5g/L （23.7~40）. The level of urine NAG was 72.3？38.6 U/g.cr （from 16~126.7） （normal？16.5U/g.cr）, and RBP was 11.3？ 17.1 mg/L （from 0.57~50.4） （normal ？0.5mg/L）. 7 patients had elevated serum creatine, 8 hypertension, and 8 had anemia （from 76~102g/L） who had no myeloma by bone marrow biopsy. Serum protein electrophoresis （SPEP） showed that only one patient had monoclonal （M） spikes （IgG？） in serum from 7 patients. 5 patients had decreased serum complement C3, while C4 normal. In histological examinations, alll patients showed proliferative glomerulonephritis, 3 of them had nodular lesions and one presented cellular and fibro-cellular crescents （33.3%）. On EM, the granular dense deposits were identified predominately in subendothelial, mesangial and occasional in subepithelial region. 2 had massive crystal lattice in subendothelial, mesangial and ocasional subepithelia regions. By IF, all patients had IgG and C3 glomerular diposits, and 5 had C1q deposits. Subtype IgG and light chain staining showed IgG3？ in 3, IgG3？ in 3, IgG1？ in 2, and IgG1？ in one. Conclusion： The clinical features of patients with PGNMID were massive proteinuria, heamaturia, renal dysfunction as well as anemia. There was predominance of IgG3-kappa glomerular deposits associated with granular dense and occasional crystal lattice deposits.
出处
《肾脏病与透析肾移植杂志》
CAS
CSCD
北大核心
2012年第5期401-407,共7页
Chinese Journal of Nephrology,Dialysis & Transplantation
