Faedda R, Pirisi M, Satta A, Bosincu L, Bartoli E
Istituto di Patologia Medica, University of Sassari, Italy.
Clin Pharmacol Ther. 1996 Nov;60(5):561-7. doi: 10.1016/S0009-9236(96)90152-6.
The aim of our study was to determine whether immunosuppressive treatment is effective in preventing and reversing the evolution of Berger's disease toward chronic renal failure.
We studied 20 unselected, consecutive patients with biopsy-proven Berger's disease who met the criteria for disease progression. They had proteinuria, significant histologic changes, persistent hematuria, and red cell casts. The treatment consisted of prednisone in an alternate-day regimen and cyclophosphamide, either in a daily oral administration or in a monthly intravenous pulse injection, both given for a 6-month cycle. Five patients had chronic renal failure (as disclosed by plasma creatinine of 230 +/- 71 mumol/L), hypertension, and proteinuria (2.7 +/- 0.8 gm/day), whereas the remaining 15 patients had normal renal function (plasma creatinine, 97 +/- 18 mumol/L) and less severe proteinuria (1.9 +/- 1.1 gm/day). However, even these 15 patients had a significant number of risk factors heralding progression to chronic renal failure.
Over an average follow-up of 8.7 +/- 3.7 years (range, 5 to 15 years), all patients but one had complete disease remission, including five patients with incipient chronic renal failure. Relapse occurred in two patients who were healed after a repeat treatment cycle. Over the entire follow-up period, no patient progressed to chronic renal failure and plasma creatinine concentration remained stable, even in subjects in whom it was high before treatment (257 +/- 79 versus 230 +/- 71 mumol/L; p > 0.05).
The immunosuppressive treatment of patients with Berger's disease with high probability of progression appears to be effective in the prevention of end-stage renal disease.
我们研究的目的是确定免疫抑制治疗在预防和逆转伯杰氏病向慢性肾衰竭发展方面是否有效。
我们研究了20例经活检证实患有伯杰氏病且符合疾病进展标准的未经挑选的连续患者。他们有蛋白尿、显著的组织学改变、持续性血尿和红细胞管型。治疗方案包括隔日服用泼尼松以及环磷酰胺，环磷酰胺采用每日口服或每月静脉脉冲注射，两种给药方式均持续6个月。5例患者患有慢性肾衰竭（血浆肌酐为230±71μmol/L）、高血压和蛋白尿（2.7±0.8g/天），而其余15例患者肾功能正常（血浆肌酐为97±18μmol/L）且蛋白尿较轻（1.9±1.1g/天）。然而，即使是这15例患者也有大量预示向慢性肾衰竭进展的危险因素。
在平均8.7±3.7年（范围为5至15年）的随访中，除1例患者外，所有患者疾病均完全缓解，包括5例早期慢性肾衰竭患者。2例经重复治疗周期治愈的患者出现复发。在整个随访期间，没有患者进展为慢性肾衰竭，血浆肌酐浓度保持稳定，即使是治疗前浓度较高的患者也是如此（257±79与230±71μmol/L；p>0.05）。
对有高度进展可能性的伯杰氏病患者进行免疫抑制治疗似乎对预防终末期肾病有效。
