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中华肾病研究电子杂志 ›› 2013, Vol. 02 ›› Issue (01) : 5 -10. doi: 10.3877/cma.j.issn.2095-3216.2013.01.002

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膜性肾病的诊断与治疗
章友康1,(), 李英2   
  1. 1.100034 北京大学第一医院肾内科
    2.河北医科大学第三医院肾内科
  • 出版日期:2013-02-15
  • 通信作者: 章友康

The diagnosis and treatment of membranous nephropathy

You-kang ZHANG1,(), Ying LI1   

  1. 1.Renal Division, Peking University First Hospital, Beijing 100034, China
  • Published:2013-02-15
  • Corresponding author: You-kang ZHANG
引用本文:

章友康, 李英. 膜性肾病的诊断与治疗[J/OL]. 中华肾病研究电子杂志, 2013, 02(01): 5-10.

You-kang ZHANG, Ying LI. The diagnosis and treatment of membranous nephropathy[J/OL]. Chinese Journal of Kidney Disease Investigation(Electronic Edition), 2013, 02(01): 5-10.

膜性肾病(MN)占原发性肾病综合征(NS)的20% ~ 35%,是国内外常见引起肾病综合症的病理类型之一。MN是一种病理学诊断,肾小球基底膜(GBM)上皮细胞侧常有多数、规则的免疫复合物为其病理学特征。依据病因可将其分为特发性膜性肾病(IMN)和继发性膜性肾病(SMN)。IMN免疫荧光常以IgG4为主,伴C3呈颗粒样沿GBM分布。若免疫荧光以IgG1和(或)IgG2 为主,并出现C1q 和(或)C4 沉积等其他情况,要认真排除SMN。目前的治疗方案主要分为保守治疗(非免疫抑制治疗),如血管紧张素转换酶抑制剂或血管紧张素Ⅱ受体拮抗剂(ACEI 或ARBs)控制血压、降低尿蛋白,纠正脂代谢紊乱,控制血糖,利尿消肿及中药等;免疫抑制治疗,如糖皮质激素、烷化剂(包括环磷酰胺、苯丁酸氮芥)、钙调神经磷酸酶抑制剂(包括环孢素A、他克莫司)等。2012年改善全球肾脏病预后(KDIGO)指南建议免疫抑制治疗首选糖皮质激素加烷化剂,推荐意大利方案,对此方案不适宜者建议选用钙调神经磷酸酶抑制剂(环孢素A、他克莫司)。此外,吗替麦考酚酯、利妥昔单抗、促肾上腺皮质激素等也用于膜性肾病的治疗,但仅有一些小样本的研究证明其有效性及安全性,缺乏大规模随机对照试验(RCT)支持。

Membranous nephropathy (MN), accounting for about 20% to 35% of primary nephrotic syndrome (NS), is one of the common pathological types for NS in the world. MN is a pathological diagnosis, characterized by frequent much regular deposits of immune complexes on the side of the epithelial cells of the glomerular basement membrane (GBM). According to the etiology, MN can be divided into idiopathic membranous nephropathy (IMN) and secondary membranous nephropathy(SMN). SMN should be carefully excluded when the immunofluorescence mainly shows IgG1 and/or IgG2 deposits together with the emergence of C1q and/or C4 deposition. At present the treatment protocols can be divided into conservative treatment (non-immunosuppressive therapy) and immunosuppressive therapy. The conservative treatment includes controlling blood pressure, decreasing proteinuria, controlling blood glucose and blood fat, inducing diuresis to reduce edema by angiotinsinconverting enzyme inhibitors/angiotinsin receptor blockers (ACEI/ARBs), and/or Chinese medicinal herbs and so on. The immunosuppressive therapy usually consists of glucocorticoids, alkylating agents (cyclophosphamide,chlorambucil), and calcineurin inhibitors (cyclosporine A, tacrolimus), etc. According to the guideline of Kidney Disease: Improving Global Outcomes (KDIGO) published in 2012, glucocorticoid plus alkylating agents was the preferred protocol. If patients have contraindication or intolerance of it, calcineurin inhibitors are suggested. Furthermore, mycophenolate mofetil (MMF), rituximab, and ACTH, etc, can also be applied to IMN, but they have been supported by just a few small-sample studies for their effectivity and safety, and will need support from large-sample randomized controlled trials in the future.

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