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不同年龄段的IgA血管炎:是时候采用精准医学方法了吗?

IgA Vasculitis Across the Ages: Is It Time for a Precision Medicine Approach?

作者信息

Gage A, Pepper R J, Marro J, Salama A D, Oni L

机构信息

Centre for Kidney and Bladder Health, University College London, London, United Kingdom.

University Hospitals Bristol and Weston NHS Foundation Trust, Bristol, United Kingdom.

出版信息

ACR Open Rheumatol. 2025 Sep;7(9):e70083. doi: 10.1002/acr2.70083.

Abstract

IgA vasculitis (IgAV; formerly Henoch-Schönlein purpura) is a systemic small vessel vasculitis most commonly affecting the skin, gut, joints, and kidneys. Nephritis is the most concerning complication for all ages because it carries the risk of progression to irreversible end-stage kidney failure. The multiorgan nature of the disease, variation in clinical presentation, and unpredictable disease course pose a substantial challenge to timely diagnosis, risk stratification, and a unified approach to management. Precision medicine is defined as a health care approach that uses genetic and molecular profiling alongside phenotypic and environmental data to generate insights to prevent or treat disease. This review article aims to provide an overview of IgAV using the latest literature to highlight three key areas in which precision medicine may have a role in advancing patient outcomes. These three areas are as follows: early phenotyping, risk stratification, and evidence-based management. Due to the presence of nephritis bringing the greatest risk of morbidity and mortality for patients with this disease, kidney involvement forms the focal point of this review. Like other forms of glomerulonephritis, there are defined stages, from diagnosis to early signs of nephritis, histologically established nephritis, chronic kidney disease, and ultimately kidney failure. Advancing a disease for which there has been very little progress provides a huge opportunity to incorporate precision medicine from the outset to augment traditional monitoring and provide more rapid evidence generation. With transformative treatments on the horizon for IgA-related glomerular diseases, improving patient outcomes to prevent kidney failure in IgAV is becoming a near reality.

摘要

IgA血管炎(IgAV;原称过敏性紫癜)是一种系统性小血管炎,最常累及皮肤、肠道、关节和肾脏。肾炎是各年龄段最令人担忧的并发症,因为它有进展为不可逆终末期肾衰竭的风险。该疾病的多器官性质、临床表现的差异以及不可预测的病程,对及时诊断、风险分层和统一的管理方法构成了重大挑战。精准医学被定义为一种医疗保健方法,它利用基因和分子图谱以及表型和环境数据来生成预防或治疗疾病的见解。这篇综述文章旨在利用最新文献对IgA血管炎进行概述,以突出精准医学可能在改善患者预后方面发挥作用的三个关键领域。这三个领域如下:早期表型分析、风险分层和循证管理。由于肾炎是该疾病患者发病和死亡风险最高的因素,肾脏受累是本综述的重点。与其他形式的肾小球肾炎一样,从诊断到肾炎的早期迹象、组织学确诊的肾炎、慢性肾脏病,直至最终肾衰竭,都有明确的阶段。对于一种进展甚微的疾病,从一开始就引入精准医学以加强传统监测并更快地生成证据,提供了巨大的机会。随着针对IgA相关肾小球疾病的变革性治疗即将出现,改善IgA血管炎患者的预后以预防肾衰竭正逐渐成为现实。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7589/12426765/3692d4b39f80/ACR2-7-e70083-g002.jpg

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