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IgA 肾小球肾炎发病机制与治疗方面的新进展有哪些。

What is new in the pathogenesis and treatment of IgA glomerulonephritis.

作者信息

Salvadori Maurizio, Rosso Giuseppina

机构信息

Department of Renal Transplantation, Careggi University Hospital, Florence 50139, Tuscany, Italy.

Division of Nephrology, San Giovanni di Dio Hospital, Florence 50143, Toscana, Italy.

出版信息

World J Nephrol. 2024 Dec 25;13(4):98709. doi: 10.5527/wjn.v13.i4.98709.

Abstract

Recently, new findings have been clarified concerning both pathogenesis and treatment of IgA nephritis. The four hits theory has been confirmed but several genetic wide association studies have allowed finding several genes connected with the pathogenesis of the disease. All these new genes apply to each of the four hits. Additionally, new discoveries concerning the microbiota and its connection with immune system and IgA generation have allowed finding out the role of the mucosa in IgA nephropathy pathogenesis. The IgA treatment is also changed included the future possibilities. The treatment of the chronic kidney disease, associated with the nephropathy, is mandatory, since the beginning of the disease. The classical immunosuppressive agents have poor effect. The corticosteroids remain an important cornerstone in any phase of the disease. More effect is related to the treatment of B cells and plasma cells. In particular, in very recent studies have been documented the efficacy of anti B cell-activating factor and anti A proliferation-inducing ligand agents. Most of these studies are to date in phase II/III. Finally, new agents targeting complement are arising. These agents also are still in randomized trials and act principally in hit 4 where the immunocomplexes in the mesangium activate the different pathways of the complement cascade.

摘要

最近,关于IgA肾病的发病机制和治疗有了新的研究发现。“四步打击”理论已得到证实,多项全基因组关联研究发现了多个与该疾病发病机制相关的基因。所有这些新基因都适用于“四步打击”中的每一步。此外,关于微生物群及其与免疫系统和IgA产生之间联系的新发现,揭示了黏膜在IgA肾病发病机制中的作用。IgA肾病的治疗也有所变化,包括未来的治疗可能性。从疾病一开始,就必须对与该肾病相关的慢性肾脏病进行治疗。传统的免疫抑制剂效果不佳。皮质类固醇在疾病的任何阶段仍然是重要的基石。更多的疗效与B细胞和浆细胞的治疗有关。特别是,最近的研究记录了抗B细胞活化因子和抗增殖诱导配体药物的疗效。迄今为止,这些研究大多处于II/III期。最后,针对补体的新型药物不断涌现。这些药物仍在进行随机试验,主要作用于第四步打击,即系膜中的免疫复合物激活补体级联反应的不同途径。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/23f9/11572654/38395bb25530/98709-g001.jpg

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