Lim Regina Shaoying, Yeo See Cheng, Barratt Jonathan, Rizk Dana V
Department of Renal Medicine, Tan Tock Seng Hospital, 11 Jalan Tan Tock Seng, Singapore 308433, Singapore.
Department of Cardiovascular Sciences, University of Leicester, Leicester LE1 7RH, UK.
J Clin Med. 2024 Feb 7;13(4):947. doi: 10.3390/jcm13040947.
Immunoglobulin A nephropathy (IgAN) remains the leading cause of primary glomerular disease worldwide. Outcomes are poor with high rates of progressive chronic kidney disease and kidney failure, which contributes to global healthcare costs. Although this disease entity has been described, there were no disease-specific treatments until recently, with the current standard of care focusing on optimal supportive measures including lifestyle modifications and optimization of the renin-angiotensin-aldosterone blockade. However, with significant advances in the understanding of the pathogenesis of IgAN in the past decade, and the acceptance of surrogate outcomes for accelerated drug approval, there have been many new investigational agents tested to target this disease. As these agents become available, we envision a multi-pronged treatment strategy that simultaneously targets the consequences of ongoing nephron loss, stopping any glomerular inflammation, inhibiting pro-fibrotic signals in the glomerulus and tubulo-interstitium, and inhibiting the production of pathogenic IgA molecules. This review is an update on a previous review published in 2021, and we aim to summarize the developments and updates in therapeutic strategies in IgAN and highlight the promising discoveries that are likely to add to our armamentarium.
免疫球蛋白A肾病（IgAN）仍是全球原发性肾小球疾病的主要病因。其预后较差，慢性肾脏病进展和肾衰竭发生率较高，这增加了全球医疗成本。尽管这种疾病已被描述，但直到最近才有针对该疾病的特异性治疗方法，目前的治疗标准侧重于最佳支持措施，包括生活方式改变和肾素 - 血管紧张素 - 醛固酮系统阻断的优化。然而，在过去十年中，随着对IgAN发病机制的理解取得重大进展，以及加速药物批准的替代结局得到认可，已经有许多新的研究药物针对该疾病进行了测试。随着这些药物的出现，我们设想了一种多管齐下的治疗策略，同时针对持续的肾单位丢失的后果、阻止任何肾小球炎症、抑制肾小球和肾小管间质中的促纤维化信号以及抑制致病性IgA分子的产生。本综述是对2021年发表的先前综述的更新，我们旨在总结IgAN治疗策略的发展和更新，并强调可能会增加我们治疗手段的有前景的发现。
