Branten A J, Wetzels J F
Department of Medicine, University Medical Center Nijmegen, The Netherlands.
Clin Nephrol. 2001 Jul;56(1):1-9.
Up to half of the patients with idiopathic membranous nephropathy (iMN) will develop renal failure. Preferably, immunosuppressive treatment should be restricted to patients at risk for the development of end-stage renal disease. However, the evidence that immunosuppressive treatment is effective in patients with iMN and renal insufficiency is weak and based on few studies with short follow-up in a limited number of patients.
We have analyzed the efficacy of immunosuppressive treatment in a large number of patients with membranous nephropathy and renal insufficiency. Since 1991, we have prospectively treated 39 patients (31 M, 8 F) with membranous nephropathy and evidence of deterioration of renal function. Treatment consisted of oral cyclophosphamide, 1.5-2.0 mg/kg body weight for 12 months, and corticosteroids for 6 months. At regular intervals blood pressure, serum creatinine, serum albumin, and proteinuria were measured. Adverse events were recorded.
Average follow-up is 32 months (range 6-104), 18 patients have been followed for more than 3 years. Mean age of the patients was 55 +/- 12 years. In the 6 months before start of therapy, serum creatinine increased from 150 +/- 74 to 226 +/- 108 micromol/l. After start of treatment renal function rapidly improved, serum creatinine at 12 months averaging 143 +/- 62 micromol/l. Proteinuria decreased from 10.3 +/- 4.9 g/10 mmol creatinine at baseline to 2.2 +/- 2.4 g/10 mmol creatinine at month 12. These initial favorable effects have persisted. Overall, 12 patients have developed a complete remission of proteinuria (persistent in 11), and an additional 19 have developed a partial remission of proteinuria (persistent in 15). Thus far, only one treated patient has developed end-stage renal disease. Side effects are a major drawback of the treatment, with 7 patients being admitted, mainly for the treatment of infectious complications.
Cyclophosphamide is effective in the treatment of patients with idiopathic membranous nephropathy and deterioration of renal function. The favorable effects are maintained well beyond the one-year treatment period. Therefore, we propose that in patients with iMN immunosuppressive therapy can be restricted to patients at high risk for end-stage renal disease.
高达半数的特发性膜性肾病(iMN)患者会发展为肾衰竭。理想情况下,免疫抑制治疗应仅限于有发展为终末期肾病风险的患者。然而,免疫抑制治疗对iMN和肾功能不全患者有效的证据薄弱,且基于对少数患者进行的随访时间较短的少量研究。
我们分析了免疫抑制治疗对大量膜性肾病和肾功能不全患者的疗效。自1991年以来,我们前瞻性地治疗了39例(31例男性,8例女性)膜性肾病且有肾功能恶化证据的患者。治疗包括口服环磷酰胺,1.5 - 2.0毫克/千克体重,持续12个月,以及使用皮质类固醇6个月。定期测量血压、血清肌酐、血清白蛋白和蛋白尿。记录不良事件。
平均随访时间为32个月(范围6 - 104个月),18例患者随访超过3年。患者的平均年龄为55±12岁。在治疗开始前的6个月,血清肌酐从150±74微摩尔/升升至226±108微摩尔/升。治疗开始后肾功能迅速改善,12个月时血清肌酐平均为143±62微摩尔/升。蛋白尿从基线时的10.3±4.9克/10毫摩尔肌酐降至第12个月时的2.2±2.4克/10毫摩尔肌酐。这些最初的良好效果持续存在。总体而言,12例患者实现了蛋白尿完全缓解(11例持续缓解),另有19例实现了蛋白尿部分缓解(15例持续缓解)。迄今为止,仅1例接受治疗的患者发展为终末期肾病。副作用是该治疗的一个主要缺点,7例患者入院,主要是为了治疗感染并发症。
环磷酰胺对治疗特发性膜性肾病和肾功能恶化患者有效。良好效果在一年治疗期后仍能很好地维持。因此,我们建议对于iMN患者,免疫抑制治疗可仅限于有高风险发展为终末期肾病的患者。