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在全身性和单一性血管炎中发生动脉和静脉血栓形成
Federica Bello1, Filippo Fagni2,3, Giacomo Bagni4
1Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy.
Nature reviews. Rheumatology
|May 6, 2025
概括
系统性血管炎和相关的遗传疾病增加了动脉或静脉血栓事件 (AVTE) 的风险,这是由于复杂的炎症和凝血相互作用. 早期诊断和结合抗血栓和免疫抑制治疗对于管理AVTE和改善患者结果至关重要.
科学领域:
- 类风湿病学 类风湿病学
- 血液学 血液学 血液学
- 免疫学 免疫学 免疫学
背景情况:
- 系统性血管炎,包括抗中性粒细胞质抗体相关的血管炎,大血管血管炎和贝赫特综合征,经常导致动脉或静脉血栓事件 (AVTE).
- 像DADA2和VEXAS综合征这样的新兴遗传疾病也存在血管性特征和AVTE的显著风险.
- 血管炎中的AVTE是由炎症和凝血通路之间的复杂相互作用引起的.
研究的目的:
- 审查系统性血管炎和相关遗传疾病中AVTE背后的机制.
- 突出AVTE的诊断挑战和预后影响.
- 讨论目前和新兴的血管炎中AVTE的管理策略.
主要方法:
- 对系统性血管炎中AVTE的病原遗传机制,临床表现和治疗策略的文献综述.
- 分析内皮功能障碍,免疫复合体沉积和细胞因子相互作用在血栓形成中的作用.
- 关于抗血栓和免疫抑制疗法的证据综合.
主要成果:
- 内皮功能障碍,免疫复合体沉积和与前血栓性因素相互作用的促炎性细胞因子在血管炎相关的AVTE中驱动血栓形成.
- AVTE显著增加疾病负担,复杂诊断,并恶化预后,导致较高的发病率和死亡率.
- 有效的管理需要解决血栓和炎症组件.
结论:
- 早期诊断和及时,全面的治疗对于预防系统性血管炎中AVTE的不可逆转损伤至关重要.
- 管理策略应基于个体风险评估整合抗血栓治疗 (如阿司匹林,抗凝剂).
- 免疫抑制疗法是基本的,特别是在诸如贝希特综合征这样的疾病中,血管炎症是血栓形成的关键驱动因素.
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