Salvadè Vanja, Teta Daniel, Guzzo Gabriella
Service de néphrologie, Centre hospitalier du Valais romand, Hôpital de Sion, 1951 Sion.
Rev Med Suisse. 2024 Feb 28;20(863):445-450. doi: 10.53738/REVMED.2024.20.863.445.
Primary IgA nephropathy is a common glomerular disease, heterogeneous in its clinical presentation. Historically considered to assume a benign course, we actually know that up to 40% of the cases progress to end stage renal disease at 20-30 years. Clinical and basic research has now allowed to understand the pathophysiology of this disease, to predict its course and to treat progressive forms more aggressively. This article summarizes classical treatments and recent therapeutic additions (sparsentan and targeted-release budenoside), which demonstrated remarkable efficacy. Complement inhibitors and B cells or plasma cells inhibitors to target aberrant IgA production have already emerged as new potential treatments. Collectively these advances may open a new therapeutic era in the management of this disease.
原发性IgA肾病是一种常见的肾小球疾病,临床表现具有异质性。过去认为其病程呈良性,但实际上我们现在知道,高达40%的病例在20至30年后会进展为终末期肾病。临床和基础研究现已使我们能够了解这种疾病的病理生理学,预测其病程,并更积极地治疗进展性形式。本文总结了经典治疗方法以及近期增加的治疗方法(司帕生坦和靶向释放布地奈德),这些方法已显示出显著疗效。针对异常IgA产生的补体抑制剂以及B细胞或浆细胞抑制剂已成为新的潜在治疗方法。总体而言,这些进展可能会开启该疾病治疗的新时代。