特发性膜性肾病研究进展.pdfVIP

108 C h inese Jou rnal of Practical n tern alM ed icine Feb. 2011 V ol. 31 No. 2 丁小强, 刘春凤 : 1005- 2194( 20 11) 02- 0 108- 05 : R5 : A : 膜性肾病是引起成人肾病综合征( N S) 的最常见病因之一, 分为特发性和继发性特发性膜性 肾病( MN ) 制不明, 多认为是与免疫 制有关的主动过程, 中性内肽酶 ( N EP )M 型磷脂酶 A2 受体 ( PLA R)醛糖还原酶( AR)和超氧化物歧化酶( SOD 2)原位抗原的发现是近期膜性肾病发病 制的重要 2 进展M N 临床过程非常多样, 随访 10 年以上的研究表明未经治疗的 M N 患者中50% ~ 60% 在 10 ~ 20 年内进展至终末期肾病( ESRD), 另有 30% 左右可以自发性缓解肾功能持续减退及持续严重蛋白尿等 是预后不佳的重要指标低危 MN 患者以对症支持治疗为主, 若合并高危因素, 则应给予特异性治疗 特异性治疗 MN 的首选方案是糖皮质激素联合细胞毒类药物, 若有禁忌或疗效不佳, 还可选择环孢素 A 他克莫司或霉酚酸酯 ( 最好联合适当剂量糖皮质激素), 也可尝试应用合成促肾上腺皮质激素R itux mi ab 等药物总之, 治疗应基于患者状况, 给予个体化治疗 : 特发性膜性肾病; 肾病综合征; 免疫抑制剂治疗 P rogress on id iopath ic m em branou s n ephropathy. D ING X iaoqiang, LIU Chunf eng. D epartm ent of N eph rology, Zhongshan H osp ital, Fudan Uni ersity, Shanghai 200032, Ch ina Summ ary: diopath ic m embranous nephropa thy ( M N) is the m ost comm on cause of nephro tic syndrome in adu lts. T he Pathog enesis of the d isease rem a ins unc lear. R ecent stud ies have detec ted som e antibodies directed a ga inst neutra l endopeptidase in new borns, M type phospho lipase2 recepto rs, a ldo se reductase( AR) and m anga nese superox ide d ism utase ( SOD2) in adu lts w ith M N. The natura l course of the d isease is v ariab le, w ith about oneth ird of patients hav ing spontaneous rem ission. H owever, fo llowup lasting mo re than 10 years reported that 50% ~ 60% of untreated pa tients died o r ended w ith endstage rena l failure. T he pe rsistence of prote inur ia and re nal dysfunction a re the r isk fac to rs of poor prognosis. Spec ific mi muno suppressive therapies shou ld be g iven to h igh risk MN patients, inc luding g luco co rtico ids, cy totox ic drugs, cyc lo spor ine ( CsA ), tacro lmi us, m ycopheno late m of e til or o thers. T o patients w ith low M N r isk, sym ptom atic treatm ent is enough. A nyhow, the the rape