Chen Yizhi, Tang Li, Feng Zhe, Cao Xueying, Sun Xuefeng, Liu Moyan, Liu Shuwen, Zhang Xueguang, Li Ping, Wei Ribao, Qiu Qiang, Cai Guangyan, Chen Xiangmei
Division of Nephrology, State Key Discipline of Internal Medicine (Nephrology), State Key Laboratory of Kidney Disease (2011DAV00088), National Clinical Medical Research Center for Kidney Disease (2013BAI09B05), Medical Quality Control Center for Kidney Disease, Chinese People's Liberation Army (PLA) General Hospital (301 Hospital), Chinese PLA Medical Academy, 28 Fuxing Road, Haidian District, Beijing, 100853, People's Republic of China.
J Nephrol. 2014 Jun;27(3):307-16. doi: 10.1007/s40620-014-0057-0. Epub 2014 Feb 13.
The outcome of idiopathic membranous nephropathy (IMN) in adults with nephrotic-range proteinuria and decreased renal function has seldom been described and the predictive value of pathological features is debated. This study aimed to describe the clinical course of this patient subgroup and to identify independently predictive pathological features.
We evaluated 129 adults with biopsy-proven IMN diagnosed from 2002 to 2011. All patients had chronic kidney disease (CKD) stages 2-4 and nephrotic-range proteinuria (≥3.5 g/day). Primary outcomes were a 20 or 50 % decline in renal function, progression to end-stage renal disease (ESRD), or all-cause mortality.
Of 129 patients, 38 (30 %) presented with proteinuria ≥8.0 g/day and 37 (29 %) with CKD stages 3-4. Thirteen (10 %) presented with segmental sclerosis, 97 (75 %) with arteriosclerosis, 42 (33 %) with moderate-to-severe tubulointerstitial injury, and 86 (67 %) with C3 deposition. Over a median follow-up of 34 months (range 12-135), 51 patients (40 %) had a 20 % decline in renal function, 27 (21 %) a 50 % decline, 14 (11 %) developed ESRD, and 19 (15 %) died. Segmental sclerosis and tubulointerstitial injury but not arteriosclerosis or C3 deposition were independent risk factors for 20 and 50 % renal function decline and progression to ESRD.
Segmental sclerosis and tubulointerstitial injury predict renal outcomes independent of clinical data in nephrotic IMN patients with decreased renal function.
关于成人特发性膜性肾病（IMN）伴肾病范围蛋白尿及肾功能减退的预后情况鲜有描述，且病理特征的预测价值存在争议。本研究旨在描述该患者亚组的临床病程，并确定具有独立预测性的病理特征。
我们评估了129例于2002年至2011年经活检证实为IMN的成人患者。所有患者均处于慢性肾脏病（CKD）2 - 4期且有肾病范围蛋白尿（≥3.5 g/天）。主要结局为肾功能下降20%或50%、进展至终末期肾病（ESRD）或全因死亡。
129例患者中，38例（30%）蛋白尿≥8.0 g/天，37例（29%）处于CKD 3 - 4期。13例（10%）出现节段性硬化，97例（75%）出现动脉硬化，42例（33%）出现中至重度肾小管间质损伤，86例（67%）出现C3沉积。中位随访34个月（范围12 - 135个月），51例患者（40%）肾功能下降20%，27例（21%）下降50%，14例（11%）进展为ESRD，19例（15%）死亡。节段性硬化和肾小管间质损伤而非动脉硬化或C3沉积是肾功能下降20%和50%以及进展至ESRD的独立危险因素。
在肾功能减退的肾病性IMN患者中，节段性硬化和肾小管间质损伤可独立于临床数据预测肾脏结局。
