Kitching A Richard, Hutton Holly L
Centre for Inflammatory Diseases, Department of Medicine, School of Clinical Sciences, Monash University, Clayton, Victoria, Australia.
Department of Nephrology, and.
Clin J Am Soc Nephrol. 2016 Sep 7;11(9):1664-1674. doi: 10.2215/CJN.13791215. Epub 2016 Apr 12.
Glomerular diseases are common and important. They can arise from systemic inflammatory or metabolic diseases that affect the kidney. Alternately, they are caused primarily by local glomerular abnormalities, including genetic diseases. Both intrinsic glomerular cells and leukocytes are critical to the healthy glomerulus and to glomerular dysregulation in disease. Mesangial cells, endothelial cells, podocytes, and parietal epithelial cells within the glomerulus all play unique and specialized roles. Although a specific disease often primarily affects a particular cell type, the close proximity, and interdependent functions and interactions between cells mean that even diseases affecting one cell type usually indirectly influence others. In addition to those cells intrinsic to the glomerulus, leukocytes patrol the glomerulus in health and mediate injury in disease. Distinct leukocyte types and subsets are present, with some being involved in different ways in an individual glomerular disease. Cells of the innate and adaptive immune systems are important, directing systemic immune and inflammatory responses, locally mediating injury, and potentially dampening inflammation and facilitating repair. The advent of new genetic and molecular techniques, and new disease models means that we better understand both the basic biology of the glomerulus and the pathogenesis of glomerular disease. This understanding should lead to better diagnostic techniques, biomarkers, and predictors of prognosis, disease severity, and relapse. With this knowledge comes the promise of better therapies in the future, directed toward halting pathways of injury and fibrosis, or interrupting the underlying pathophysiology of the individual diseases that lead to significant and progressive glomerular disease.
肾小球疾病常见且重要。它们可源于影响肾脏的全身性炎症或代谢性疾病。或者，它们主要由局部肾小球异常引起，包括遗传性疾病。肾小球内在细胞和白细胞对于健康的肾小球以及疾病中的肾小球调节异常都至关重要。肾小球内的系膜细胞、内皮细胞、足细胞和壁层上皮细胞都发挥着独特而专门的作用。尽管特定疾病通常主要影响特定细胞类型，但细胞之间的紧密 proximity、相互依存的功能和相互作用意味着即使是影响一种细胞类型的疾病通常也会间接影响其他细胞类型。除了肾小球固有的那些细胞外，白细胞在健康状态下巡逻肾小球，并在疾病中介导损伤。存在不同类型和亚群的白细胞，其中一些以不同方式参与个体肾小球疾病。先天性和适应性免疫系统的细胞很重要，它们指导全身性免疫和炎症反应，在局部介导损伤，并可能减轻炎症和促进修复。新的遗传和分子技术以及新的疾病模型的出现意味着我们更好地理解了肾小球的基础生物学和肾小球疾病的发病机制。这种理解应该会带来更好的诊断技术、生物标志物以及预后、疾病严重程度和复发的预测指标。有了这些知识，未来就有望出现更好的治疗方法，旨在阻断损伤和纤维化途径，或中断导致严重和进行性肾小球疾病的个体疾病的潜在病理生理学。 （注：原文中“proximity”拼写有误，正确拼写为“proximity”，意为“接近，临近” ）
