Moura Lúcio R R, Franco Marcello F, Kirsztajn Gianna Mastroianni
Hospital Israelita Albert Einstein, Brazil.
Universidade Federal de São Paulo, Brazil.
J Bras Nefrol. 2015 Oct-Dec;37(4):475-80. doi: 10.5935/0101-2800.20150075.
There is scarce data on the clinical profile of adult Brazilian patients with nephrotic syndrome caused by minimal change disease (MCD) and focal segmental glomerulosclerosis.
We evaluated the clinical characteristics and response to treatment in adult patients with nephrotic syndrome having a histological diagnosis of MCD or FSGS.
This is a retrospective analysis of 50 patients with MCD and 120 with FSGS. All patients were initially treated with steroids. The study outcomes were: steroid responsiveness, prevalence of total remission, progression to chronic renal failure and need of renal replacement therapy due to end-stage renal disease (ESRD).
Initial serum creatinine level was 24% higher among patients with FSGS (p = 0.02), and proteinuria levels were 36% higher in MCD (p < 0.001). Patients with MCD were sensitive to steroid therapy in 80% of the cases, with total remission in 74%, while patients with FSGS were sensitive in 58% (p = 0.01), with total remission in 30% (p = 0.002). Patients with FSGS had an acute renal failure prevalence of 39% (vs. 12%, p = 0.013) and ESRD of 10% (vs. 0%, p < 0.001). Steroid responsiveness reduced in 83% the risk of ESRD (p < 0.001), while total remission was associated to a reduction in risk of 89% (p < 0.001).
A positive response to steroid therapy was the most important factor related with preservation of renal function and FSGS was related with less steroid responsiveness.
关于巴西成年微小病变病(MCD)和局灶节段性肾小球硬化所致肾病综合征患者的临床特征,相关数据匮乏。
我们评估了经组织学诊断为MCD或FSGS的成年肾病综合征患者的临床特征及治疗反应。
这是一项对50例MCD患者和120例FSGS患者的回顾性分析。所有患者最初均接受类固醇治疗。研究结果包括:类固醇反应性、完全缓解率、进展为慢性肾衰竭以及因终末期肾病(ESRD)而需要肾脏替代治疗的情况。
FSGS患者的初始血清肌酐水平高出24%(p = 0.02),而MCD患者的蛋白尿水平高出36%(p < 0.001)。MCD患者80%对类固醇治疗敏感,完全缓解率为74%,而FSGS患者58%敏感(p = 0.01),完全缓解率为30%(p = 0.002)。FSGS患者急性肾衰竭患病率为39%(相比之下为12%,p = 0.013),ESRD患病率为10%(相比之下为0%,p < 0.001)。类固醇反应性使ESRD风险降低83%(p < 0.001),而完全缓解与风险降低89%相关(p < 0.001)。
对类固醇治疗的阳性反应是与肾功能保留相关的最重要因素,而FSGS与类固醇反应性较低相关。