Floege Jürgen, Bernier-Jean Amelie, Barratt Jonathan, Rovin Brad
Department of Nephrology and Department of Cardiology, RWTH Aachen University Hospital, Aachen, Germany.
Department of Medicine, University of Montreal, Montreal, Québec, Canada; Nephrology Service, CIUSSS du Nord-de-l'Île-de-Montreal, Montreal, Québec, Canada.
Kidney Int. 2025 Apr;107(4):640-651. doi: 10.1016/j.kint.2025.01.014. Epub 2025 Jan 31.
IgA nephropathy (IgAN), the world's most common primary glomerular disease, carries a significant lifetime risk for kidney failure as well as an enormous socioeconomic burden. In the past, studies in patients with IgAN largely focused on optimizing so-called supportive care, that is, blockade of the renin-angiotensin system, blood pressure control, and lifestyle modifications. The effectiveness of immunosuppressive measures, particularly high-dose corticosteroid therapy, has been reported variably, but there is considerable evidence for an increase in serious adverse effects with such therapies. This disappointing situation has changed dramatically with a better understanding of the pathogenesis of IgAN, and with regulatory agencies accepting changes in proteinuria and the estimated glomerular filtration rate loss or slope over 2 to 3 years as surrogate outcome markers. A multitude of new therapies are now being evaluated in IgAN, and several drugs, such as sodium-glucose transporter-2 inhibitors, sparsentan (a dual endothelin-1 and angiotensin II receptor blocker), nefecon (a targeted release formulation of budesonide), and iptacopan (a complement factor B inhibitor), have been approved, with more to come in the next few years. In this review, we propose a new treatment paradigm that combines therapies with different mechanisms of action to target the immune components and the chronic kidney disease components of IgAN in parallel to preserve long-term kidney survival.
IgA肾病(IgAN)是全球最常见的原发性肾小球疾病,终生面临肾衰竭的重大风险,同时带来巨大的社会经济负担。过去,针对IgA肾病患者的研究主要集中在优化所谓的支持性治疗,即阻断肾素-血管紧张素系统、控制血压和改变生活方式。免疫抑制措施的有效性,尤其是高剂量皮质类固醇治疗的有效性,报道不一,但有大量证据表明此类治疗会增加严重不良反应。随着对IgA肾病发病机制的深入了解,以及监管机构将2至3年内蛋白尿的变化和估计的肾小球滤过率降低或下降斜率作为替代结局指标,这种令人失望的情况已发生巨大变化。目前,多种新疗法正在IgA肾病中进行评估,几种药物,如钠-葡萄糖转运蛋白2抑制剂、司帕生坦(一种双重内皮素-1和血管紧张素II受体阻滞剂)、奈非那酮(一种布地奈德靶向释放制剂)和依他库帕(一种补体因子B抑制剂)已获批准,未来几年还会有更多药物获批。在本综述中,我们提出一种新的治疗模式,将具有不同作用机制的疗法结合起来,同时针对IgA肾病的免疫成分和慢性肾脏病成分,以维持长期肾脏存活。