Faedda R, Pirisi M, Satta A, Tanda F, Bartoli E
Istituto di Patologia Medica, Università degli Studi, Sassari, Italy.
Clin Nephrol. 1996 Oct;46(4):237-44.
To assess the effectiveness of an intensive immunosuppressive regimen on the nephrotic syndrome due to mixed membranous and mesangial lesions, we studied 18 patients with nephrotic syndrome and miscellaneous histologic features characterized by mesangial proliferation and sclerosis, non-specific basement membrane changes such as thickening, fraying and scalloping, in the absence of extensive immune complex deposition by immunofluorescence. The patients were treated with an immunosuppressive regimen that combined prednisone and cyclophosphamide for at least 6 months with the following schedule: 1) induction with prednisone daily 250 to 750 mg i.v. for 3 to 8 days, plus cyclophosphamide 100 to 200 mg p.o. daily; 2) maintenance with prednisone 100 to 200 mg p.o. in alternate days for 30 to 75 days, and cyclophosphamide as before; 3) tapering, with prednisone in alternate day regimen, reduced on average by 25 mg every month, plus cyclophosphamide as before; 4) discontinuation of cyclophosphamide and slow withdrawal of prednisone. Treatment lasted on average 9 months, with an average cumulative dose of prednisone of 9.2 g and of cyclophosphamide of 26.7 g. At the end of treatment, 14 patients had a complete remission and 4 remained stable. On longer follow-up, one out of these 4 patients, who had renal failure before treatment, subsequently progressed to end-stage renal disease. Nine patients relapsed after an average remission of 6 years. Eight of them remitted completely on a repeat cycle. One patient refused the retreatment and progressed to end-stage renal disease within one year. After an average follow-up of 7.3 +/- 1.1 years, plasma creatinine for the whole group had fallen from 138 +/- 26 to 103 +/- 20 mumol/l and proteinuria from 6.7 +/- 0.7 to 0.4 +/- 0.2 g/d (p < 0.001). In conclusion, in patients with these forms of nephrotic syndrome this immunosuppressive regimen is highly effective in inducing remission, in preventing progression to end-stage renal disease and in treating relapses.
为评估强化免疫抑制方案对混合性膜性和系膜性病变所致肾病综合征的疗效，我们研究了18例具有系膜增生和硬化、非特异性基底膜改变（如增厚、磨损和呈扇形）等多种组织学特征的肾病综合征患者，免疫荧光检查未发现广泛免疫复合物沉积。患者接受免疫抑制方案治疗，该方案联合使用泼尼松和环磷酰胺至少6个月，具体方案如下：1）诱导期：静脉注射泼尼松每日250至750毫克，持续3至8天，加口服环磷酰胺每日100至200毫克；2）维持期：隔日口服泼尼松100至200毫克，持续30至75天，环磷酰胺用量同前；3）减量期：采用隔日泼尼松方案，平均每月减少25毫克，环磷酰胺用量同前；4）停用环磷酰胺，缓慢停用泼尼松。治疗平均持续9个月，泼尼松平均累积剂量为9.2克，环磷酰胺平均累积剂量为26.7克。治疗结束时，14例患者完全缓解，4例病情稳定。在更长时间的随访中，这4例患者中有1例在治疗前就已出现肾衰竭，随后进展为终末期肾病。9例患者在平均缓解6年后复发。其中8例在重复治疗周期后完全缓解。1例患者拒绝再次治疗，1年内进展为终末期肾病。经过平均7.3±1.1年的随访，全组患者的血浆肌酐从138±26降至103±20微摩尔/升，蛋白尿从6.7±0.7降至0.4±0.2克/天（p<0.001）。总之，对于这些类型的肾病综合征患者，这种免疫抑制方案在诱导缓解、预防进展为终末期肾病以及治疗复发方面非常有效。
