在VEXAS综合征中对真菌细菌感染的敏感性
Stanislas Riescher1, Raphael Lecomte2, Gwenvael Danic1
1Service de Médecine Interne, CHU de Nantes, Nantes, France.
Rheumatology (Oxford, England)
|February 5, 2024
概括
患有VEXAS综合征的患者,一种新发现的自身炎症性疾病,容易受到传播的非结核性真菌菌感染. 在VEXAS患者中,对机会性感染的早期怀疑至关重要,以防止严重的结果.
科学领域:
- 血液学 血液学 血液学
- 免疫学 免疫学 免疫学
- 传染性疾病 传染性疾病
背景情况:
- 维克萨斯 (VEXAS) 综合征 (真空,E1酶,X链接,自身炎症,体质) 是一种由UBA1.1.体质突变引起的获得性自身炎症和血液学疾病.
- 目前,VEXAS并未被认为是获得性免疫缺陷的原因.
研究的目的:
- 调查VEXAS与传播的非结核性真菌菌感染 (NTMi) 之间的关联.
- 了解VEXAS患者NTMi的临床特征和结果.
主要方法:
- 在13年间对患有扩散NTMi的患者进行了回顾性研究.
- 包括标准包括对Mycobacterium avium的阳性培养物和对20个传播的NTMi病例的审查.
主要成果:
- 在十名VEXAS患者中,有两名患上了传播的Mycobacterium avium感染.
- 在患有NTMi的HIV阴性患者中,有两个患有VEXAS;两人都在两年内死亡.
- 在七个NTMi病例中观察到单细胞减小.
结论:
- 维克萨斯患者对传播的NTMi表现出敏感性,可能是由于单细胞功能障碍.
- NTMi可以模仿VEXAS火焰,因此需要高度怀疑机会性感染.
- 需要进一步的研究来阐明VEXAS和NTMi之间的关系.
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