罕见病研究2024,Vol.3Issue(4) :423-430.DOI:10.12376/j.issn.2097-0501.2024.04.003

自身炎症性疾病相关肾脏损害

Renal Impairment Associated with Autoinflammatory Diseases

简珊 王长燕 张彩慧 宋红梅
罕见病研究2024,Vol.3Issue(4) :423-430.DOI:10.12376/j.issn.2097-0501.2024.04.003

自身炎症性疾病相关肾脏损害

Renal Impairment Associated with Autoinflammatory Diseases

简珊 1王长燕 1张彩慧 1宋红梅1
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作者信息

  • 1. 中国医学科学院北京协和医院儿科,北京 100730
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摘要

肾脏是自身炎症性疾病(AIDs)的重要受累靶器官之一.AIDs相关肾脏损害的临床表现多种多样,可表现为血尿、蛋白尿、肾病综合征、高血压、肾动脉狭窄、肾功能不全等.发病机制主要包括肾淀粉样变性和(或)由炎症小体激活导致的肾血管炎/血管病变等.肾脏受累与否及受累程度与AIDs患者的预后密切相关.本文通过介绍AIDs相关肾脏损害的发病机制、部分AIDs相关肾损害的临床特点等,旨在提高对AIDs相关肾脏损害的认识,早期诊断、早期治疗,以提高患者的生活质量,改善预后.

Abstract

Kidney is one of the key target organs involved in autoinflammatory diseases(AIDs).The clinical manifestations of renal impairment associated with AIDs are diverse,including hematuria,proteinuria,nephrotic syndrome,hypertension,renal artery stenosis,renal insufficiency,and others.The pathogenesis is mostly renal amyloidosis and/or renal vasculitis/vasculopathy caused by inflammasome activation.Whether or not the kidney is involved and its degree is closely related to the prognosis of AIDs patients.This article intro-duces the pathogenesis of AIDs-related renal impairmen and highlights the clinical characteristics of renal dam-age in some AIDs,aiming to enhance clinicians'understanding of AIDs-related renal damage,and to implement early diagnosis and treatment to improve patients'quality of life and prognosis.

关键词

自身炎症性疾病/肾脏损害/AA型淀粉样变性/血管炎

Key words

autoinflammatory diseases/kidney impairment/AA amyloidosis/vasculitis

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出版年

2024
罕见病研究

罕见病研究

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