Department of Nephrology, Addenbrooke's Hospital, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK.
Br J Hosp Med (Lond). 2024 Jul 30;85(7):1-11. doi: 10.12968/hmed.2024.0044.
Primary glomerulonephritis comprises several renal-limited diseases that can cause haematoproteinuria, chronic kidney disease, nephrosis and end stage kidney disease. The most common of these are IgA nephropathy (IgAN), primary membranous nephropathy (PMN), Focal Segmental Glomerulosclerosis (FSGS) and Minimal Change Disease (MCD). Although rare, these diseases cause a significant burden to health care systems, given the high cost of treating End Stage Kidney Disease (ESKD) with dialysis or transplantation. Until recently, the pathogenesis of primary gloerulonephritis has remained obscure. However, recent advances in understanding of how these diseases evolve has led to the introduction of novel therapeutic agents. Trials are underway or have recently completed that have huge implications for the standard of care for the primary glomerulonephritidies, and should dramatically reduce the number of patients who progress onto end stage kidney disease. This article reviews the international Kidney Disease Improving Global Outcomes (KDIGO) guidelines for the treatment of IgAN, PMN, FSGS and MCD, as well as recent research on pathogenesis and treatment.
原发性肾小球肾炎包括几种肾脏局限性疾病,可导致血红蛋白尿、慢性肾脏病、肾病综合征和终末期肾病。其中最常见的是 IgA 肾病(IgAN)、原发性膜性肾病(PMN)、局灶节段性肾小球硬化症(FSGS)和微小病变性肾病(MCD)。尽管这些疾病很少见,但由于透析或移植治疗终末期肾病(ESKD)的成本很高,它们给医疗保健系统带来了巨大的负担。直到最近,原发性肾小球肾炎的发病机制仍不清楚。然而,近年来对这些疾病如何演变的理解的进展,导致了新型治疗药物的引入。正在进行或最近已经完成的试验对原发性肾小球肾炎的治疗标准具有巨大影响,应该会大大减少进展为终末期肾病的患者数量。本文回顾了国际肾脏病改善全球结局组织(KDIGO)对 IgAN、PMN、FSGS 和 MCD 的治疗指南,以及发病机制和治疗的最新研究。