Wendt Ralph, Sobhani Alina, Diefenhardt Paul, Trappe Moritz, Völker Linus Alexander
Department of Nephrology, Hospital St. Georg Leipzig, Delitzscher Str. 141, 04129 Leipzig, Germany.
Department II of Internal Medicine, Center for Molecular Medicine Cologne, Faculty of Medicine, University Hospital Cologne, University of Cologne, 50937 Cologne, Germany.
Biomedicines. 2024 Oct 4;12(10):2259. doi: 10.3390/biomedicines12102259.
There have been exciting advances in our knowledge of primary glomerular diseases and nephrotic syndromes in recent years. Beyond the histological pattern from renal biopsy, more precise phenotyping of the diseases and the use of modern nephrogenetics helps to improve treatment decisions and sometimes also avoid unnecessary exposure to potentially toxic immunosuppression. New biomarkers have led to easier and more accurate diagnoses and more targeted therapeutic decisions. The treatment landscape is becoming wider with a pipeline of promising new therapeutic agents with more sophisticated approaches. This review focuses on all aspects of entities that are associated with nephrotic syndromes with updated information on recent advances in each field. This includes podocytopathies (focal segmental glomerulosclerosis and minimal-change disease), membranous nephropathy, membranoproliferative glomerulonephritis, IgA nephropathy, fibrillary glomerulonephritis, amyloidosis, and monoclonal gammopathy of renal significance in the context of the nephrotic syndrome, but also renal involvement in systemic diseases, diabetic nephropathy, and drugs that are associated with nephrotic syndromes.
近年来，我们对原发性肾小球疾病和肾病综合征的认识取得了令人振奋的进展。除了肾活检的组织学模式外，对疾病进行更精确的表型分析以及运用现代肾脏遗传学有助于改善治疗决策，有时还能避免不必要地暴露于潜在有毒的免疫抑制治疗中。新的生物标志物使诊断更加简便准确，治疗决策更具针对性。随着一系列采用更复杂方法的有前景的新型治疗药物的出现，治疗方案日益丰富。本综述聚焦于与肾病综合征相关的各个方面，并提供每个领域最新进展的更新信息。这包括足细胞病（局灶节段性肾小球硬化和微小病变病）、膜性肾病、膜增生性肾小球肾炎、IgA肾病、纤维样肾小球肾炎、淀粉样变性以及肾病综合征背景下具有肾脏意义的单克隆丙种球蛋白病，还包括系统性疾病中的肾脏受累、糖尿病肾病以及与肾病综合征相关的药物。
