Section of Nephrology, Department of Internal Medicine, Rush University Medical Center, Chicago, Illinois, USA.
J Am Soc Nephrol. 2012 Nov;23(11):1769-76. doi: 10.1681/ASN.2012040389. Epub 2012 Sep 20.
Over the last 20 years, primary FSGS has emerged as one of the leading causes of idiopathic nephrotic syndrome in adults, particularly among African Americans. In nephrotic patients, progression to ESRD often occurs over the course of 5-10 years, whereas non-nephrotic patients and those entering a remission have an extremely favorable prognosis. As a result, it is in patients who remain persistently nephrotic despite conservative therapy that a more aggressive therapeutic approach is taken. Primary FSGS was once considered an entity nonresponsive to prednisone or immunosuppressive agents, but it has become apparent over the last 20 years that a substantial portion of nephrotic adults with primary FSGS do respond to treatment with a significantly improved prognosis. The recent histologic classification proposed for FSGS has provided additional insights into the prognosis and response to therapy. This article reviews the current knowledge regarding the presentation, prognosis, and therapeutic approach in adults with primary FSGS.
在过去的 20 年中，原发性 FSGS 已成为成人特发性肾病综合征的主要病因之一，尤其是在非裔美国人中。在肾病综合征患者中，进展为终末期肾病（ESRD）通常需要 5-10 年的时间，而非肾病综合征患者和那些进入缓解期的患者具有极好的预后。因此，在那些尽管采用保守治疗仍持续肾病综合征的患者中，会采取更积极的治疗方法。原发性 FSGS 曾被认为对泼尼松或免疫抑制剂无反应，但在过去的 20 年中，大量原发性 FSGS 的肾病综合征成人患者对治疗有反应，预后明显改善。最近提出的 FSGS 组织学分类为预后和治疗反应提供了更多的见解。本文综述了原发性 FSGS 成人患者的临床表现、预后和治疗方法的最新知识。
