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与VEXAS综合征和慢性骨髓单细胞白血病相关的结节性多炎:一个病例对照研究

Paul Cioni¹, Yann Nguyen², Julien Rohmer³

¹Department of Internal Medicine, Cochin University Hospital, Paris, France.

Rheumatology (Oxford, England)

|December 24, 2025

概括

与VEXAS综合征或CMML相关的多关节炎 (PAN) 呈现不同,并且比初级PAN更糟糕的结果. 早期识别和量身定制的管理对于改善患者在这些不同的条件的预后至关重要.

科学领域:

类风湿病学类风湿病学
血液学血液学血液学
内部医学内部医学

背景情况:

多关节炎 (PAN) 是一种罕见的全身性死性血管炎.
PAN可以独立发生 (原发性PAN) 或与其他疾病相关,如VEXAS综合征和慢性骨髓单细胞白血病 (CMML).

研究的目的:

将VEXAS相关PAN (VEXAS-PAN) 和CMML相关PAN (CMML-PAN) 的临床表现,结果和治疗特征与初级PAN进行比较.
在管理二次形式的PAN时识别独特的特征和挑战.

主要方法:

一项回顾性多中心研究,涉及诊断为初级PAN,VEXAS-PAN或CMML-PAN的患者.
根据年龄和性别,VEXAS-PAN和CMML-PAN病例与三个主要PAN病例的匹配.
分析基线特征,治疗反应,生存率和复发率.

主要成果:

二次性PAN (VEXAS-PAN和CMML-PAN) 与初级PAN相比,发生在显著较大的年龄.
VEXAS-PAN显示皮肤,耳炎和眼部表现的发病率更高,而CMML-PAN在临床上类似于初级PAN.
与初级PAN相比,VEXAS-PAN和CMML-PAN的缓解率较低,复发率更高. 在VEXAS-PAN.中,死亡率明显较高.

结论:

维克萨斯-PAN和CMML-PAN代表着不同的临床实体,具有独特的表现和较差的预后.
这些二次形式的PAN带来了特定的治疗挑战,需要个性化的管理策略.
早期识别和量身定制的治疗对于改善VEXAS或CMML相关PAN患者的治疗结果至关重要.

关键词:

慢性骨髓单细胞白血病 (CMML) 是一种疾病.多关节炎结节性 (PAN)维克萨斯综合征 (VEXAS综合征) 是一种血管炎是一种血管炎.

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