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| Ⅰ型干扰素相关肾病 |
| 康梅子1,2,刘亚伟1,3,戴兵1* |
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(1. 海军军医大学(第二军医大学)第二附属医院肾内科, 解放军肾脏病研究所, 上海 200003;
2. 南通大学第二附属医院肾内科, 南通 226001;
3. 海军军医大学(第二军医大学)第二附属医院内科教研室, 上海 200003
*通信作者) |
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| 摘要: |
| 当机体发生病毒感染、自身免疫异常和1型干扰素病等疾病时常常会出现增强的Ⅰ型干扰素（IFN-Ⅰ）信号转导通路活化，所有这些与IFN-Ⅰ活化相关的疾病均可导致肾脏损伤并有着相似的组织病理学特征。其机制既可以通过IFN-Ⅰ直接损伤肾脏足细胞、系膜细胞、内皮细胞和壁层上皮细胞，也可以通过IFN-Ⅰ活化诱发免疫反应介导肾小球损伤，这一类肾脏疾病被命名为IFN-Ⅰ相关肾病。本文对IFN-Ⅰ相关肾病的发病机制、病因分类、组织病理学特征、诊断及治疗作一综述。 |
| 关键词: 塌陷性肾小球病变 Ⅰ型干扰素 系统性红斑狼疮 病毒感染 血栓性微血管病 |
| DOI：10.16781/j.CN31-2187/R.20220423 |
| 投稿时间：2022-05-18修订日期：2023-04-23 |
| 基金项目:国家自然科学基金(81970640). |
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| Type Ⅰ interferon-related kidney disorders |
| KANG Meizi1,2,LIU Yawei1,3,DAI Bing1* |
(1. Department of Nephrology, Kidney Disease Institute of PLA, The Second Affiliated Hospital of Naval Medical University(Second Military Medical University), Shanghai 200003, China;
2. Department of Nephrology, The Second Affiliated Hospital of Nantong University, Nantong 226001, Jiangsu, China;
3. Department of Internal Medicine, The Second Affiliated Hospital of Naval Medical University(Second Military Medical University), Shanghai 200003, China
*Corresponding author) |
| Abstract: |
| Enhanced type Ⅰ interferon (IFN-Ⅰ) signaling pathway is observed in the setting of viral infections, autoimmune abnormality, type 1 interferonopathies, etc. All these IFN-Ⅰ-related disorders can cause renal dysfunction and share common histopathological features. The underlying mechanism lies in that IFN-Ⅰ can mediate kidney injury either by directly damaging several renal cell types (including podocytes, mesangial, endothelial and parietal epithelial cells) or promoting immune-mediated glomerular injury. This emerging spectrum of kidney diseases is defined as IFN-Ⅰ-related kidney disorders. This paper reviews the pathogenesis, etiological classification, histopathological characteristics, and diagnosis and treatment of the diseases. |
| Key words: collapsing glomerulopathy interferon-Ⅰ systemic lupus erythematosus viral infections thrombotic microangiopathy |
