最新常見成人原發性腎病症候群致病機轉及治療

Update on Mechanisms and Management of Common Idiopathic Nephrotic Syndrome in Adults

郭嘉文(Chia-Wen Kuo) ； 陳金順(Jin-Shuen Chen)

《內科學誌》 24卷5期 (2013/10) Pp. 377-387

https://doi.org/10.6314/JIMT.2013.24(5).03

摘要

全球末期腎病變進入透析患者與日俱增，在臺灣亦是如此。根據台灣腎臟醫學會資料，造成末期腎病變最常見的原因依序為腎絲球疾病、糖尿病腎臟病及高血壓性腎病變。其中，腎絲球疾病依臨床表現可區分診斷為腎炎症候群或腎病症候群。腎病症候群為一綜合表現的診斷須包含蛋白尿、低白蛋白血症、水腫及血脂肪過高。造成的原因可以來自原發性及續發性原因，其中原發性約占80%。而臺灣最常見成人原發性腎病症候群包含有：微小腎絲球疾病、膜性腎絲球病變及局部節段型腎絲球硬化症。近年來對此三大原發性疾病的研究有頗多進展。另外Kidney Disease：Improving Global Outcome這一全球性非營利機構，也於2012揭櫫新的治療原則。故本文章將著重於此些病生理機轉上的新發展及對新的治療原則做一介紹。

關鍵字

腎病症候群 ； 微小腎絲球疾病 ； 膜性腎絲球病變 ； 局部節段型腎絲球硬化

並列摘要

End stage renal disease (ESRD) and dialysis dependence is increasing all over the world. Taiwan has the same problem. According to data from the Taiwan Society of Nephrology, the most common cause of ESRD is glomerulonephritis (GN), followed by diabetic nephropathy, and hypertensive nephropathy. GN can be classified into nephritic and nephrotic syndrome according to the clinical presentations. Nephrotic syndrome is diagnosed as a complex of clinical manifestations including proteinuria, hypoalbuminemia, edema and hyperlipidemia. The etiologies can be divided into secondary causes and primary causes. The primary causes account for 80% of all cases. The three most common causes of adult idiopathic nephrotic syndrome (INS) are minimal change disease, membranous nephropathy and focal segmental glomerulosclerosis. There has been a rapid increase in studies of these three INS in recent years, and the Kidney Disease: Improving Global Outcomes (KDIGO), a global non-profit foundation, declared the practicing guidelines for these three INS in 2012. In this article, we will focus on the new developments of the pathophysiologies and introduce new management guidelines.

並列關鍵字

Nephrotic syndrome ； Minimal change disease ； Membranous nephropathy ； Focal segmental glomerulosclerosis