特发性膜性肾病研究进展-新医学.PDFVIP

108 C inese Journal of Practical InternalM edicine Feb. 2011 Vol. 31 No. 2 丁小强, 刘春凤 : 1005- 2194( 2011) 02- 0108- 05 : R5 : A : 膜性肾病是引起成人肾病综合征( NS)的最常见病因之一, 分为特发性和继发性特发性膜性 肾病( IMN )机制不明, 多认为是与免疫机制有关的主动过程, 中性内肽酶 ( NEP)M 型磷脂酶 A 受体 2 ( PLA R)醛糖还原酶(AR)和超氧化物歧化酶( SOD2)原位抗原的发现是近期膜性肾病发病机制的重要 2 进展IMN 临床过程非常多样, 随访 10年以上的研究表明未经治疗的 IMN 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 IMN 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 IMN 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 ituxmi 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 Progress on idiopathicm em ranousnephropathy. DING X iao2qiang, LIU Chun2f eng. D epartm ent of N eph2 rology, Zhongshan H osp ital, Fudan University, Shanghai 200032, C ina Summ ary: Idiopat ic m embranous nep ropat y ( IMN) is t e m ost comm on cause of nep rotic syndrome in adu lts. T e Pat ogenesis of t e d isease rem ains unclear. Recent stud ies ave detected som e antibodies directed a2 gainst neutral endopeptidase in new borns, M2type p osp olipase22 receptors, aldose reductase( AR) and m anga2 nese superoxide d ism utase ( SOD2) in adu ltsw it IMN. T e natural course of t e d isease is variab le, w it about one2t ird of patients aving spontaneous rem ission. H owever, follow2up lasting more t an 10 years reported t at 50% ~ 60% of untreated patients died or endedw it end2stage renal failure. T e persistence of proteinuria and re2 nal dysfunction are t e risk factors of poor prognosis. Specific mi munosuppressive t erapies s ou ld be given to ig risk IMN patients, including glucocorticoids, cytotoxic drugs, cyclosporine ( CsA ), tacrolmi us, m ycop enolate m of2 etil or ot ers. To patientsw it low IMN risk, sym ptom atic treatm ent is enoug . Any ow, t e t erapeutic re