中国血液净化

中国血液净化 ›› 2017, Vol. 16 ›› Issue (04): 242-246.doi: 10.3969/j.issn.1671-4091.2017.04.007

• 临床研究 • 上一篇    下一篇

年轻人膜性肾病的临床病理特点及治疗效果

郭艳红1,郑丹侠1,王悦1   

  1. 1. 北京大学第三医院
  • 收稿日期:2016-12-01 修回日期:2017-02-15 出版日期:2017-04-12 发布日期:2017-04-12
  • 通讯作者: 王悦 bjwangyue@sina.com E-mail:bjwangyue@sina.com

The clinicopathological and prognostic features of young patients with membranous nephropathy

  • Received:2016-12-01 Revised:2017-02-15 Online:2017-04-12 Published:2017-04-12

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摘要： 目的探讨年轻人膜性肾病的临床病理特点以及治疗效果。方法纳入2000～2016 年于北京大学第三医院肾内科住院患者中15～30 岁、肾活检证实为膜性肾病、临床表现为肾病综合征的52 例患者作为病例组，随机选取同时期40～60 岁表现为肾病综合征的膜性肾病患者53 例作为对照组，分析其临床病理特点以及治疗效果。结果①临床上，年轻患者肾穿刺时24h 尿蛋白、血浆白蛋白与中老年组相比无显著差异[(9.7±4.8)g 比(9.4±4.6)g，t=0.294，P=0.770；(24.5±5.7)g/L 比(24.4±3.6)g/L，t=0.100，P=0.921]，高血压的比例(1.9%比37.7%，χ2=21.040，P＜0.001)、镜下血尿比例(44.2%比67.9%，χ2=5.986，P=0.014)、血肌酐水平[(64.9±20.4)μmol/L 比(77.0±31.7)μmol/L，t=- 2.311，P=0.023]均显著降低，eGFR 的水平显著升高[(150.7±44.4)ml/min 比(104.6±31.0)ml/min，t=6.145，P＜
0.001]，从起病到确诊的时间显著缩短[4.0(2.0，12.0)周比12.0(4.0，32.0)周，Z=-3.184，P=0.001]。②病理上，年轻患者不典型膜性肾病占51.9%，中老年组患者不典型膜性肾病占9.4%，差异显著(51.9%比9.4% ，χ2=26.785，P＜0.001)；年轻患者荧光染色IgA、IgM 的阳性比例显著高于中老年组(32.7% 比11.3%，χ2=7.008，P=0.008；51.9%比24.5%，χ2=8.353，P=0.004)，肾小管间质病变、肾小血管病变积分显著低于中老年组[0.0(0.0，1.0)比1.0(0.0，2.0)，Z=-4.102，P＜0.001；0.0(0.0，1.0)比1.0(1.0，1.0)，Z= -3.984，P＜0.001]。③治疗上，年轻膜性肾病患者中48.1%的患者单用激素治疗，51.9%的患者采用激素联合免疫抑制剂治疗，中老年组100%的患者采用激素联合免疫抑制剂治疗；年轻患者中单用激素治疗与激素联合环磷酰胺治疗相比，治疗完全缓解率、部分缓解率、复发率以及从治疗至部分缓解和完全缓解的时间均未见统计学差异(28.0%比18.2%，χ2=0.629，P=0.428；36.0%比63.6%，χ2=3.577，P=0.059；7.7%比18.5%，χ2=3.225，P=0.199；(2.5±1.5)月比(3.0±1.5)月，t=-1.094，P=0.281；(5.0±2.8)月比(7.4±2.0)月，t=-1.818，P=0.087)；年轻患者激素联合环磷酰胺治疗与中老年组激素联合环磷酰胺治疗相比，两组的完全缓解率、部分缓解率以及复发率均无统计学差异(18.2%比35.8%，χ2=2.282，P=0.131；63.6%比43.4%, χ2=2.548, P=0.110；9.1%比9.4%，χ2=0.002，P=0.963)，年轻患者达到部分缓解以及完全缓解的时间显著短于中老年患者[(3.0±1.5)月比(5.3±5.0)月，t=-2.594, P=0.012；(7.4±2.0)月比(12.0±7.0)月，t=-3.081，P=0.005]。结论就表现为肾病综合征的膜性肾病患者而言，年轻人与中老年相比确诊时肾病综合征程度、治疗缓解率和复发率大致相似，但年轻患者起病至确诊时间较短，高血压、镜下血尿比例较低，eGFR 较高，不典型膜性肾病比例较高，肾小管间质病变及肾小血管病变较轻，治疗起效较快，部分患者对单独激素治疗有效。

关键词: 膜性肾病, 年轻患者

Abstract: Objective To investigate the clinicopathological and prognostic features of young patients with membranous nephropathy (MN). Methods The clinical data of 52 young patients (15-30 years old) diagnosed as MN and nephrotic syndrome and admitted to Peking University Third Hospital from 2000 to 2016 were retrospectively analyzed. Comparison was made between these patients and the 53 patients of 40～60 years old diagnosed as MN and nephrotic syndrome during the same period in the hospital. Clinicopathological and prognostic features of the two groups of patients were analyzed. Results ①Clinically, there were no differences in 24 hour urinary protein and serum albumin (9.7±4.8g vs. 9.4±4.6g, t=0.294, P=0.770 for urinary protein; 24.5±5.7g/L vs. 24.4±3.6g/L, t=0.100, P=0.921 for serum albumin) between the two groups. However, young MN patients had lower proportion of hypertension (1.9% vs. 37.7%, χ2=21.040, P＜0.001), lower proportion of microscopical hematuria (44.2% vs. 67.9%, χ2=5.986, P=0.014), lower serum creatinine (64.9±20.4μmol/L vs. 77.0 ± 31.7μmol/L, t=- 2.311, P=0.023), higher estimated glomerular filtration rate (eGFR) (150.7±44.4ml/min vs. 104.6±31.0ml/min, t=6.145, P＜0.001) and shorter disease course [4.0(2.0, 12.0)w vs. 12.0(4.0, 32.0)w, Z=-3.184, P=0.001]. ②Pathologically, more atypical MN features were present in young patients (51.9% vs. 9.4%, χ2=26.785, P＜0.001), and stage II was the main pathological change in older patients. Higher positive ratios of IgA (32.7% vs. 11.3%, χ2=7.008, P=0.008) and IgM (51.9% vs. 24.5%, χ2=8.353, P= 0.004), less renal tubulointerstitial lesions [0.0(0.0, 1.0) vs. 1.0(0.0, 2.0), Z=-4.102, P＜0.001] and less arterial wall thickening [0.0(0.0, 1.0) vs. 1.0(1.0, 1.0), Z=-3.984, P＜0.001] were the characteristics of young patients. ③ Therapeutically, 48.1% young patients received corticosteroid monotherapy, and 51.9% young patients received corticosteroids plus immunosuppressants. In the older group, all patients received corticosteroids plus immunosuppressants. In the young group, there were no differences in partial remission rate, complete remission rate, recurrent rate, partial remission time and complete remission time (28.0% vs. 18.2%, χ2=0.629, P=0.428; 36.0% vs. 63.6%, χ2=3.577, P=0.059; 7.7% vs. 18.5%, χ2=3.225, P=0.199; 2.5±1.5m vs. 3.0±1.5m, t=-1.094, P=0.281; 5.0±2.8m vs. 7.4±2.0m, t=-1.818, P=0.087) between the patients with corticosteroid monotherapy and those with corticosteroids plus cyclophosphamide. There were no significant differences in partial remission rate, complete remission rate and recurrent rate between the young patients with corticosteroids plus cyclophosphamide and the older patients with corticosteroids plus cyclophosphamide (18.2% vs. 35.8%, χ2=2.282, P=0.131; 63.6% vs. 43.4%, χ2=2.548, P=0.110; 9.1% vs. 9.4%, χ2=0.002, P=0.963). The time from the beginning of treatment to partial remission or complete remission was shorter in the young group than in the older group (3.0±1.5m vs. 5.3±5.0m, t=-2.594, P=0.012; 7.4±2.0m vs. 12±7m, t=-3.081, P=0.005). Conclusion The severity of nephrotic syndrome at the onset of the disease showed no difference between the young patients and the older patients. Young patients had lower proportion of hypertension and microscopical hematuria, higher estimated glomerular filtration rate and higher proportion of atypical MN. There were less arterial wall thickening and renal tubulointerstitial lesions in young patients. No difference in proportion of partial remission or complete remission existed between the two groups. The onset time was shorter in the young group than in the older patients. Corticosteroid monotherapy was effective to some of the young patients.

Key words: Membranous nephropathy, Young patient