关键词: 冷球蛋白血症相关肾小球肾炎, 临床特点, 病理特征, 预后

Abstract:

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 IgG 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.