特发性膜性肾病研究进展

Progress on idiopathic membranous nephropathy

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A2受体(PLA2R)、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、Rituximab等药物。总之,治疗应基于患者状况,给予个体化治疗。

Summary ：Idiopathic membranous nephropathy （IMN） is the most common cause of nephrotic syndrome in adults. The Pathogenesis of the disease remains unclear. Recent studies have detected some antibodies directed a- gainst neutral endopeptidase in newborns, M-type phospholipase-2 receptors, aldose reductase （AR） and manga- nese superoxide dismutase （SOD2） in adults with IMN. The natural course of the disease is variable, with about one-third of patients having spontaneous remission. However, follow-up lasting more than 10 years reported that 50% - 60% of untreated patients died or ended with end-stage renal failure. The persistence of proteinuria and renal dysfunction are the risk factors of poor prognosis. Specific immunosuppressive therapies should be given to high risk IMN patients, including glucoeorticoids, cytotoxic drugs, cyclosporine （ CsA ）, tacrolimus, mycophenolate mofetil or otbers. To patients with low IMN risk, symptomatic treatment is enough. Anyhow, the therapeutic regimens of IMN should based on individual cases.