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免疫介导性肾小球疾病的标准免疫抑制治疗。

Standard immunosuppressive therapy of immune-mediated glomerular diseases.

机构信息

Nefrologia, Dialisi e Trapianto, Azienda Ospedaliera G. Brotzu, Cagliari, Italy.

出版信息

Autoimmun Rev. 2013 Jun;12(8):848-53. doi: 10.1016/j.autrev.2012.11.012. Epub 2012 Dec 3.

Abstract

Glomerulonephritis (GN) accounts for 10%-20% of the total incident cases of end stage renal disease (ESRD), and is the third most common cause of ESRD after diabetes and hypertension in western countries. The pathogenesis of glomerulonephritis is prevalently immune mediated: humoral and cell-mediated immunity are involved, although the rationale for an etiological treatment is still lacking. In the last forty years, empirical treatment based upon the use of corticosteroids and/or immunosuppressive drugs have obtained excellent results in improving survival of both the patient and the kidney. Almost 95% of children affected by minimal change disease (MCD) achieve remission of proteinuria within 4 to 8weeks of prednisone administration. In adults with focal segmental glomerulosclerosis (FSGS), prednisone induces complete or partial remission in the majority of patients, but a longer period of steroid treatment or the combination of calcineurin inhibitors or cytotoxic drugs can be needed. A percentage of 65%-70% of patients with idiopathic membranous nephropathy (MN) reach complete or partial remission with a 6-month course of therapy alternating glucocorticoids with alkylating agents. Glucocorticoids plus cyclophosphamide, and, on occasion, plasmapheresis are effective in 70%-90% of patients with ANCA-associated vasculitis (AAV). Fifty percent of responders relapse within the 3-5years and currently, the mortality of AAV at 1year exceeds 15%. This article is aimed to analyze the risk-to-benefit balance of steroids and conventional immunosuppressive regimens, focusing, for a sake of brevity, on idiopathic nephrotic syndrome (INS) and ANCA associated vasculitis.

摘要

肾小球肾炎(GN)占终末期肾病(ESRD)总发病例的 10%-20%,是西方国家继糖尿病和高血压之后导致 ESRD 的第三大常见病因。肾小球肾炎的发病机制普遍为免疫介导:涉及体液和细胞免疫,尽管针对病因的治疗仍缺乏依据。在过去的四十年中,基于使用皮质类固醇和/或免疫抑制剂的经验性治疗在改善患者和肾脏的生存方面取得了极好的效果。几乎 95%的微小病变病(MCD)患儿在接受泼尼松治疗后 4-8 周内蛋白尿缓解。在局灶节段性肾小球硬化症(FSGS)的成人中,泼尼松可使大多数患者完全或部分缓解,但可能需要更长时间的类固醇治疗或联合钙调神经磷酸酶抑制剂或细胞毒性药物。特发性膜性肾病(MN)患者中有 65%-70%的患者在接受 6 个月的交替使用糖皮质激素和烷化剂治疗后完全或部分缓解。糖皮质激素加环磷酰胺,偶尔加血浆置换,对 70%-90%的抗中性粒细胞胞浆抗体相关性血管炎(AAV)患者有效。50%的缓解者在 3-5 年内复发,目前,AAV 的 1 年死亡率超过 15%。本文旨在分析类固醇和常规免疫抑制方案的风险-效益平衡,为简洁起见,重点分析特发性肾病综合征(INS)和抗中性粒细胞胞浆抗体相关性血管炎。

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