增生性肾小球肾炎伴单克隆免疫球蛋白沉积患者的临床病理特点及预后OA北大核心CSTPCD

Objective To analyze the clinicopathological features,treatment and prognosis of patients with proliferative glomerulonephritis with monoclonal immunoglobulin deposition.Methods The clinical data,renal pathology,treatment and prognosis of patients with PGNMID diagnosed by renal biopsy in Xijing Hospital from January 2018 to January 2024 were retrospectively analyzed and summarized.Results A total of 28 patients were enrolled in this study,with a male to female ratio of 4:3.The clinical manifestations were proteinuria(100%),hematuria(82.1%),renal insufficiency(28.6%),and low complement C3 or C4 emia(32.1%).One patient(3.6%)had serum monoclonal immunoglobulin,and 5 patients(17.9%)had abnormal serum free light chain ratio.The deposition of immunoglobulin in the kidney was IgG3 in 24 cases,IgG1 in 4 cases,and IgG3κwas the most common type(17 cases).Then there are 24 cases(85.7%)of MPGN,3 cases(10.7%)characterized by EPGN,1 case(3.6%)of MGN.Under electron microscope,most of the electron-dense deposits were found in the mesangial area and subendothelium,and a few were accompanied by subepithelial deposits.The follow-up time ranged from 3 to 56 months.Until the last follow-up,a total of 5 patients entered ESRD,of which 2 patients died,1 patient underwent peritoneal dialysis,1 patient underwent hemodialysis,and 1 patient underwent preparation for renal replacement therapy.Follow-up of 1 year,2 years,3 years renal total response rate of 32.1%,57.1%and 64.2%respectively.According to the situation of kidney ease into remission group(n=16)and non-remission group(n=12).The average age of the remission group was 44±17.35 years,including 8 males(50%);The average age of the non-remission group was 59.83±18.09 years,including 8 males(66.7%).The age,nT-proBNP,troponin I and urea nitrogen of the non-remission group were higher than those of the remission group,and the differences were statistically significant(P＜0.05).Among the 5 patients with renal complete remission,2 were treated with BD(bortezomib combined with dexamethasone),1 was treated with Dara,and the remaining 2 were treated with steroids combined with immunosuppressants.Conclusions The clinical manifestations of PGNMID are proteinuria,hematuria and renal insufficiency.Under light microscope,MPGN was the main manifestation,and IgG3κwas the most common subtype.Based on bortezomib treatment can obtain good hematology and kidney remission rate.Dara may be a safe and effective drug for the treatment of PGNMID,the best treatment needs further exploration.