单用泼尼松或联合吗替麦考酚酯治疗C3肾小球肾炎的疗效
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摘要
目的:探索两种方案治疗C3肾小球肾炎(C3GN)的疗效。方法:回顾性分析东部战区总医院国家肾脏疾病临床医学研究中心经肾活检明确诊断为C3GN的病例,比较泼尼松联合吗替麦考酚酯(Pred+MMF)与单用泼尼松(Pred)两种治疗方案肾脏缓解率和预后的差别。结果:(1)Pred+MMF治疗组8例,男女比例3∶5,中位年龄19岁(16～27岁),Pred治疗组19例,男女比例11∶8,中位年龄33岁(19～45岁)。两组基线临床资料和组织形态学特点无统计学差异;(2)Pred+MMF组中位随访时间43月,肾脏缓解率37.5%,1例进展至随访终点,Pred组中位随访时间41.5月,肾脏缓解率52.9%,2例进展至随访终点;两组肾脏缓解率和预后均无统计学差异;(3)11例进行了补体相关抗体检测,仅1例C3肾炎因子阳性,3例行补体相关基因检测,未发现突变。(4)两组分别有1例出现感染。结论:Pred+MMF对部分C3GN治疗有效,但疗效并不优于单纯Pred治疗。
Objective:To evaluate the therapeutic efficacy of prednisone with mycophenolate mofetil(Pred+MMF) and prednisone(Pred) alone in patients with C3 glomerulonephritis(C3 GN). Methodology:we conducted a retrospective analysis of patients with C3 GN confirmed by renal biopsy,8 patients received Pred+MMF treatment,19 patients received Pred treatment.We compared remission and prognosis of two groups. Results:(1) there were 3 males and 5 females with median age of 19(16～27) years old in Pred+MMF group.11 males and 8 females were treated with Pred alone,with the median age of 33(19～45) years old.There were no significant differences in baseline clinical data and histomorphological features between the two groups.(2) The median follow-up time was 43.5% in the Pred+MMF group,and the renal remission rate was 37.5%.One patient reached the primary endpoint.The median follow-up time in the Pred group was 41.5 months.The renal remission rate was 52.9%,and 2 patients reached the primary endpoint.There was no significant difference in renal remission rate and prognosis between the two groups.(3) 1/11 patients were positive for C3 Nef.Three patients underwent genetic testing,none of them with genetic variants in complement genes(4) There were few adverse reactions of immunosuppressive agents,and one patient in each group had infection. Conclusion:Pred+MMF is effective in the treatment of some C3 GN,but the curative effect is not better than prednisone regimen alone.
Objective:To evaluate the therapeutic efficacy of prednisone with mycophenolate mofetil(Pred+MMF) and prednisone(Pred) alone in patients with C3 glomerulonephritis(C3 GN). Methodology:we conducted a retrospective analysis of patients with C3 GN confirmed by renal biopsy,8 patients received Pred+MMF treatment,19 patients received Pred treatment.We compared remission and prognosis of two groups. Results:(1) there were 3 males and 5 females with median age of 19(16～27) years old in Pred+MMF group.11 males and 8 females were treated with Pred alone,with the median age of 33(19～45) years old.There were no significant differences in baseline clinical data and histomorphological features between the two groups.(2) The median follow-up time was 43.5% in the Pred+MMF group,and the renal remission rate was 37.5%.One patient reached the primary endpoint.The median follow-up time in the Pred group was 41.5 months.The renal remission rate was 52.9%,and 2 patients reached the primary endpoint.There was no significant difference in renal remission rate and prognosis between the two groups.(3) 1/11 patients were positive for C3 Nef.Three patients underwent genetic testing,none of them with genetic variants in complement genes(4) There were few adverse reactions of immunosuppressive agents,and one patient in each group had infection. Conclusion:Pred+MMF is effective in the treatment of some C3 GN,but the curative effect is not better than prednisone regimen alone.
引文
1 Pickering MC,D′Agati VD,Nester CM,et al.C3 glomerulopathy:consensus report.Kidney Int,2013,84(6):1079-1089.
2 Hou J,Markowitz GS,Bomback AS,et al.Toward a working definition of C3 glomerulopathy by immunofluorescence.Kidney Int,2014,85(2):450-456.
3 Barbour TD,Pickering MC,Cook HT.Recent insights into C3 glomerulopathy.Nephrol Dial Transplant,2013,28(7):1685-1693.
4 Xiao X,Pickering MC,Smith RJ.C3 glomerulopathy:the genetic and clinical findings in dense deposit disease and C3 glomerulonephritis.Semin Thromb Hemost,2014,40(4):465-471.
5 Sethi S,Fervenza FC,Zhang Y,et al.C3 glomerulonephritis:clinicopathological findings,complement abnormalities,glomerular proteomic profile,treatment,and follow-up.Kidney Int,2012,82(4):465-473.
6 Medjeral-Thomas NR,O′Shaughnessy MM,O′Regan JA,et al.C3 glomerulopathy:clinicopathologic features and predictors of outcome.Clin J Am Soc Nephrol,2014,9(1):46-53.
7 Cook HT,Pickering MC.Histopathology of MPGN and C3 glomerulopathies.Nat Rev Nephrol,2015,11(1):14-22.
8 Viswanathan GK,Nada R,Kumar A,et al.Clinico-pathologic spectrum of C3 glomerulopathy-an Indian experience.Diagn Pathol,2015,10:16.
9 Bomback AS,Smith RJ,Barile GR,et al.Eculizumab for dense deposit disease and C3 glomerulonephritis.Clin J Am Soc Nephrol,2012,7(5):748-756.
10 Nester CM,Smith RJ.Treatment options for C3 glomerulopathy.Curr Opin Nephrol Hypertens,2013,22(2):231-237.
11 Zhang Y,Nester CM,Holanda DG,et al.Soluble CR1 therapy improves complement regulation in C3 glomerulopathy.J Am Soc Nephrol,2013,24(11):1820-1829.
12 Vivarelli M,Emma F.Treatment of C3 glomerulopathy with complement blockers.Semin Thromb Hemost,2014,40(4):472-477.
13 Giaime P,Daniel L,Burtey S.Remission of C3 glomerulopathy with rituximab as only immunosuppressive therapy.Clin Nephrol,2015,83(1):57-60.
14 Haffner K,Michelfelder S,Pohl M.Successful therapy of C3Nef-positive C3 glomerulopathy with plasma therapy and immunosuppression.Pediatr Nephrol,2015,30(11):1951-1959.
15 Rabasco C,Cavero T,Roman E,et al.Effectiveness of mycophenolate mofetil in C3 glomerulonephritis.Kidney Int,2015,88(5):1153-1160.
16 Goodship TH,Cook HT,Fakhouri F,et al.Atypical hemolytic uremic syndrome and C3 glomerulopathy:conclusions from a “Kidney Disease:Improving Global Outcomes” (KDIGO) Controversies Conference.Kidney Int,2017,91(3):539-551.
17 Avasare RS,Canetta PA,Bomback AS,et al.Mycophenolate Mofetil in Combination with Steroids for Treatment of C3 Glomerulopathy:A Case Series.Clin J Am Soc Nephrol,2018,13(3):406-413.
18 Caliskan Y,Torun ES,Tiryaki TO,et al.Immunosuppressive Treatment in C3 Glomerulopathy:Is it Really Effective? Am J Nephrol,2017,46(2):96-107.
19 Ravindran A,Fervenza FC,Smith RJH,et al.C3 Glomerulopathy:Ten Years′ Experience at Mayo Clinic.Mayo Clin Proc,2018,93(8):991-1008.
20 王金泉.C3肾小球病的致病机制和对策.解放军医学杂志,2014,39(11):918-923.
2 Hou J,Markowitz GS,Bomback AS,et al.Toward a working definition of C3 glomerulopathy by immunofluorescence.Kidney Int,2014,85(2):450-456.
3 Barbour TD,Pickering MC,Cook HT.Recent insights into C3 glomerulopathy.Nephrol Dial Transplant,2013,28(7):1685-1693.
4 Xiao X,Pickering MC,Smith RJ.C3 glomerulopathy:the genetic and clinical findings in dense deposit disease and C3 glomerulonephritis.Semin Thromb Hemost,2014,40(4):465-471.
5 Sethi S,Fervenza FC,Zhang Y,et al.C3 glomerulonephritis:clinicopathological findings,complement abnormalities,glomerular proteomic profile,treatment,and follow-up.Kidney Int,2012,82(4):465-473.
6 Medjeral-Thomas NR,O′Shaughnessy MM,O′Regan JA,et al.C3 glomerulopathy:clinicopathologic features and predictors of outcome.Clin J Am Soc Nephrol,2014,9(1):46-53.
7 Cook HT,Pickering MC.Histopathology of MPGN and C3 glomerulopathies.Nat Rev Nephrol,2015,11(1):14-22.
8 Viswanathan GK,Nada R,Kumar A,et al.Clinico-pathologic spectrum of C3 glomerulopathy-an Indian experience.Diagn Pathol,2015,10:16.
9 Bomback AS,Smith RJ,Barile GR,et al.Eculizumab for dense deposit disease and C3 glomerulonephritis.Clin J Am Soc Nephrol,2012,7(5):748-756.
10 Nester CM,Smith RJ.Treatment options for C3 glomerulopathy.Curr Opin Nephrol Hypertens,2013,22(2):231-237.
11 Zhang Y,Nester CM,Holanda DG,et al.Soluble CR1 therapy improves complement regulation in C3 glomerulopathy.J Am Soc Nephrol,2013,24(11):1820-1829.
12 Vivarelli M,Emma F.Treatment of C3 glomerulopathy with complement blockers.Semin Thromb Hemost,2014,40(4):472-477.
13 Giaime P,Daniel L,Burtey S.Remission of C3 glomerulopathy with rituximab as only immunosuppressive therapy.Clin Nephrol,2015,83(1):57-60.
14 Haffner K,Michelfelder S,Pohl M.Successful therapy of C3Nef-positive C3 glomerulopathy with plasma therapy and immunosuppression.Pediatr Nephrol,2015,30(11):1951-1959.
15 Rabasco C,Cavero T,Roman E,et al.Effectiveness of mycophenolate mofetil in C3 glomerulonephritis.Kidney Int,2015,88(5):1153-1160.
16 Goodship TH,Cook HT,Fakhouri F,et al.Atypical hemolytic uremic syndrome and C3 glomerulopathy:conclusions from a “Kidney Disease:Improving Global Outcomes” (KDIGO) Controversies Conference.Kidney Int,2017,91(3):539-551.
17 Avasare RS,Canetta PA,Bomback AS,et al.Mycophenolate Mofetil in Combination with Steroids for Treatment of C3 Glomerulopathy:A Case Series.Clin J Am Soc Nephrol,2018,13(3):406-413.
18 Caliskan Y,Torun ES,Tiryaki TO,et al.Immunosuppressive Treatment in C3 Glomerulopathy:Is it Really Effective? Am J Nephrol,2017,46(2):96-107.
19 Ravindran A,Fervenza FC,Smith RJH,et al.C3 Glomerulopathy:Ten Years′ Experience at Mayo Clinic.Mayo Clin Proc,2018,93(8):991-1008.
20 王金泉.C3肾小球病的致病机制和对策.解放军医学杂志,2014,39(11):918-923.