Division of Nephrology, Department of Medicine, Columbia University College of Physicians and Surgeons, New York, NY.
Division of Nephrology, Department of Medicine, Columbia University College of Physicians and Surgeons, New York, NY.
Am J Kidney Dis. 2020 Jun;75(6):955-964. doi: 10.1053/j.ajkd.2019.12.019. Epub 2020 Apr 21.
Podocyte injury is the initiating step in the pathway toward clinically evident forms of nephrotic syndrome known as podocytopathies, represented as either minimal change disease (MCD) or focal segmental glomerulosclerosis (FSGS). There are hallmark differences in the histologic appearances of MCD and FSGS, which in turn represent distinct pathogenic models after initial podocyte injury (eg, no change in podocyte number in MCD vs podocyte detachment and death in FSGS). However, MCD and FSGS also share a number of common causes, supporting the theory that these diseases lie along a shared podocytopathy spectrum. In this installment of AJKD's Core Curriculum in Nephrology, we demonstrate how the podocytopathies can be classified according to pathogenesis and treatment response as an alternative to histologic description. Using case examples, we show how these alternative classification schemes can assist not only diagnosis, but also long-term management of podocytopathies.
足细胞损伤是向临床明显的肾病综合征形式发展的起始步骤，这些形式被称为足细胞病，表现为微小病变性肾病（MCD）或局灶节段性肾小球硬化症（FSGS）。MCD 和 FSGS 在组织学表现上存在显著差异，这反过来代表了初始足细胞损伤后的不同发病模型（例如，MCD 中足细胞数量没有变化，而 FSGS 中足细胞脱落和死亡）。然而，MCD 和 FSGS 也有许多共同的病因，这支持了这些疾病沿着共同的足细胞病谱发展的理论。在 AJKD 的肾脏病学核心课程的这一部分中，我们展示了如何根据发病机制和治疗反应对足细胞病进行分类，作为对组织学描述的替代方法。我们使用病例示例展示了这些替代分类方案不仅如何有助于诊断，而且还如何有助于足细胞病的长期管理。
