Division of Nephrology, Icahn School of Medicine at Mount Sinai, New York, NY.
Department of Pathology, Icahn School of Medicine at Mount Sinai, New York, NY.
Adv Kidney Dis Health. 2024 Jul;31(4):275-289. doi: 10.1053/j.akdh.2024.03.009.
Focal segmental glomerular sclerosis (FSGS) is a histological lesion characterized by sclerosis in sections (segmental) of some glomeruli (focal) in association with podocyte injury. Historically, FSGS has often been characterized as a disease, but it is a heterogeneous entity based on etiology, clinical course, and therapeutic approach. A unifying feature is podocyte injury and loss, which can be primary or the result of secondary maladaptive responses to glomerular stressors. FSGS has been demonstrated over time to carry a large health burden and remains a leading glomerular cause of ESRD globally. Recent clinical practice guidelines highlight the unmet scientific need for better understanding of disease pathogenesis, particularly for immunologic etiologies, as well as more targeted therapeutic drug development. In this review, we will discuss the current FSGS classification scheme, pathophysiologic mechanisms of injury, and treatment guidelines, along with emerging and investigational therapeutics.
局灶节段性肾小球硬化症(FSGS)是一种组织学病变,其特征是部分肾小球(局灶)节段发生硬化,同时伴有足细胞损伤。从历史上看,FSGS 常被定义为一种疾病,但它是一种基于病因、临床病程和治疗方法的异质性实体。一个统一的特征是足细胞损伤和丢失,它可以是原发性的,也可以是肾小球应激源的继发性适应性反应的结果。随着时间的推移,FSGS 已被证明对健康造成了巨大负担,并且仍然是全球导致终末期肾病的主要肾小球疾病之一。最近的临床实践指南强调了科学界对更好地了解疾病发病机制的迫切需求,特别是对免疫病因学的了解,以及更有针对性的治疗药物的开发。在这篇综述中,我们将讨论当前的 FSGS 分类方案、损伤的病理生理机制以及治疗指南,以及新兴和正在研究的治疗方法。