冷球蛋白血症相关肾小球肾炎患者的临床病理特征及预后分析

Clinicopathological features in patients with cryoglobulinemic glomerulonephritis

目的:分析冷球蛋白血症相关肾小球肾炎(CGN)患者的临床病理特征及预后。方法:回顾分析CGN患者的临床、病理及随访资料,Kaplan-Meier法分析肾脏存活率,COX回归模型分析预后危险因素。结果:40例CGN患者,男性24例、女性16例,肾活检平均年龄47.8±14.9岁。高血压、贫血、肾功能不全、肾病范围蛋白尿和镜下血尿发生率分别为70%、75%、57.5%、57.5%和95%。按血清是否存在单克隆免疫球蛋白(MIg)及是否伴HBV或HCV感染分为四组,即仅血清MIg(MIg)组,仅HBV或HCV感染组(HBV/HCV组),血清MIg伴HBV或HCV感染组(MIg+HBV/HCV组),MIg/HBV/HCV阴性组。MIg组肾功能不全发生率显著高于HBV/HCV组;血肌酐显著高于HBV/HCV组及MIg/HBV/HCV阴性组,与MIg+HBV/HCV组无统计学差异。常见病理类型为膜增生性肾小球肾炎(MPGN)(92.5%),MIg组肾小管萎缩/间质纤维化显著重于其余三组。肾组织多种免疫球蛋白沉积32例,单一免疫球蛋白沉积8例,单一Ig G亚型5例(其中2例单一轻链)。39例患者中位随访24.9月,中位肾脏存活时间22.1月,各组肾脏存活时间无显著差异,11例(28.9%)进入终末期肾病(ESRD),MIg组进入ESRD比率最高,但仅与HBV/HCV组有统计差异。多因素COX回归分析显示血清MIg阳性是影响CGN患者肾脏预后的独立危险因素。结论:MIg/HBV/HCV阴性的CGN最常见。MIg阳性患者肾功能不全发生率和血肌酐较高,进入ESRD较多见。血清MIg阳性是CGN患者肾脏预后的独立危险因素。血清MIg与肾组织免疫球蛋白沉积并不完全平行。

Objective： To investigate the clinicopathological features and prognosis in patients with cryoglobulinemic glomerulonephritis（ CGN）. Methodology：Forty patients with CGN fulfilled the inclusion criteria were recruited in this retrospective study.According to whether serum monoclonal immunoglobulin（ MIg） positive and/or HBV/HCV infection,forty patients were divided into four groups： MIg,HBV/HCV,MIg ＋ HBV/HCV and MIg/HBV/HCV negative group.Their clinical,pathologic and follow-up data were collected and analyzed. Results： There were 24 males and 16 females with a mean age of 47. 8 years old. Hypertension was presented in 70% of cases,anemia in 75%,renal insufficiency in 57. 5%,nephrotic proteinuria in 57. 5% and microscopic hematuria in 95%. The prevalence of renal insufficiency in MIg group was significantly higher than that in HBV/HCV group. The serum creatinine（ SCr） were significantly higher than that in HBV/HCV and MIg/HBV/HCV negative group. Membranoproliferative GN（ 92. 5%） was the predominant histologic pattern. MIg group had the most severe chronic tubular injury. Glomerular deposits of multiple immunoglobulin,single immunoglobulin and single Ig G subtype were observed in 32,8 and 5 patients,respectively.A total 39 patients had a median follow-up of 24. 9 months,and 11（ 28. 9%） patients progressed to end stage renal disease（ ESRD）.The median renal survivals were 22. 1 months.There were no significant differences of renal survivals in all the groups. MIg group had higher ESRD incidence of progressing to than HBV/HCV group. Univariate and multivariate COX analysis indicated the serum MIg positive predicting wores prognosis. Conclusion： CGN with MIg/HBV/HCV negative was the most common group.CGN patients with serum MIg positive alone had worse renal dysfunction and higher levels of SCr.Serum MIg positive was the independently prognostic factor. The serum MIg was not always consistent with the immunoglobulin deposition in the glomerulus.