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急性损伤,VEXAS综合征的一个未被认可的特征
Kambiz Kalantari1, Megan M Sullivan2, Loren P Herrera Hernandez3
1Department of Internal Medicine, Division of Nephrology, Mayo Clinic, Rochester, MN, USA.
Rheumatology (Oxford, England)
|August 26, 2024
概括
急性损伤 (AKI) 影响了25%的患有空腔,E1酶,X链,自身炎症,体质 (VEXAS) 综合征的患者. 年龄和C反应蛋白是AKI的危险因素,AKI经常复发,在活检上显示出富含血细胞的间歇性炎.
科学领域:
- 腎臟病學 (nephrology) 是一種醫學專業.
- 类风湿病学 类风湿病学
- 遗传学 遗传学 是一个
背景情况:
- 空腔,E1酶,X链接,自身炎症,体质 (VEXAS) 综合征是一种多系统的自身炎症性疾病.
- 在VEXAS综合征中,脏的参与还没有得到很好的定义.
研究的目的:
- 调查VEXAS综合征中急性损伤 (AKI) 的发生率,危险因素和组织病理特征.
- 在这个患者群体中描述AKI的临床过程和结果.
主要方法:
- 对具有基因确认UBA1突变的患者进行了回顾性图表审查.
- 考克斯回归分析以确定AKI的风险因素.
- 来自受影响患者的脏活检的组织病理学审查.
主要成果:
- 在81名患者中,有25%患有AKI,复发率为90%.
- 诊断时的年龄和高C反应蛋白 (CRP) 与AKI风险增加有关.
- 细胞活检最常见的是显示了富含血细胞的间歇性炎.
结论:
- AKI是维克萨斯综合征的一个显著而未被认可的并发症.
- 早期识别风险因素,如年龄和CRP可以帮助管理.
- 富含等离子细胞的间歇性炎是VEXAS相关AKI的关键组织病理发现.
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