Rasche Franz Maximilian, Klotz Christoph Hubert, Czock David, Karges Wolfram, Muche Rainer, Jehle Peter Michael, Mertz Andreas, Keller Frieder
Division of Nephrology, Department of Internal Medicine II, University of Ulm, Ulm, Germany.
Nephron Clin Pract. 2003;93(4):c131-6. doi: 10.1159/000070232.
For advanced progressive primary IgA nephropathy (IgAN) no established therapy exists. We conducted a prospective, uncontrolled trial to evaluate the effect of intravenous cyclophosphamide pulse (CyP) therapy on the course of advanced progressive IgAN. Twenty-one patients (mean age 52 +/- 10 years; male/female 20/1) with biopsy-proven IgAN without crescentic extracapillary proliferation and a serum creatinine of more than 2.0 mg/dl and/or an increase more than 25% in the previous 3 months were included. Patients were treated with CyP (750 mg/m(2 )body surface area) every 4 weeks for 6 months and low dose oral prednisolone. The loss of renal function per year was significantly reduced from 16% before therapy to 4% after therapy (p < 0.001). A further increase >25% of serum creatinine after therapy was observed in 8 patients after 0.7 years (range 0.3-3.0 years), and 3 of these patients developed end-stage renal disease. Proteinuria decreased significantly during CyP therapy. A low nadir of white blood cell and platelet count was associated with a better renal outcome (p = 0.025). In conclusion, CyP therapy and low dose oral prednisolone is effective in preserving renal function in a subgroup of patients with advanced progressive IgAN.
对于晚期进行性原发性IgA肾病（IgAN），目前尚无既定的治疗方法。我们进行了一项前瞻性、非对照试验，以评估静脉注射环磷酰胺脉冲疗法（CyP）对晚期进行性IgAN病程的影响。纳入了21例患者（平均年龄52±10岁；男/女为20/1），经活检证实为IgAN，无新月体性毛细血管外增生，血清肌酐超过2.0mg/dl和/或在过去3个月内升高超过25%。患者每4周接受一次CyP治疗（750mg/m²体表面积），共6个月，并服用低剂量口服泼尼松龙。每年的肾功能丧失率从治疗前的16%显著降低至治疗后的4%（p<0.001）。治疗后0.7年（范围0.3 - 3.0年），8例患者血清肌酐进一步升高>25%，其中3例患者发展为终末期肾病。在CyP治疗期间蛋白尿显著减少。白细胞和血小板计数的低最低点与较好的肾脏预后相关（p = 0.025）。总之，CyP疗法和低剂量口服泼尼松龙对晚期进行性IgAN患者亚组的肾功能具有保护作用。
