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与抗MDA5抗体相关的间歇性肺病的临床,放射和病理特征

Xixia Chen¹, Wei Jiang², Qiwen Jin¹

¹Peking University China-Japan Friendship School of Clinical Medicine, Beijing, China.

|May 31, 2023

概括

间歇性肺病 (ILD) 在抗黑色素瘤分化相关的基因5抗体阳性皮肤肌炎 (anti-MDA5+DM) 中很常见,组织性肺炎 (OP) 是主要的放射性模式. 某些模式表明预后不佳.

科学领域:

类风湿病学类风湿病学
肺部病理学肺部病理学
病理学病理学病理学

背景情况:

间歇性肺病 (ILD) 是患有抗黑色素瘤分化关联基因5抗体阳性皮肤肌炎 (anti-MDA5+DM) 患者的显著并发症.
了解这种人群中ILD的临床,放射和病理特征对于诊断和管理至关重要.

研究的目的:

调查抗黑色素瘤分化相关基因5抗体阳性皮肤肌炎 (抗MDA5+DM) 患者间歇性肺病 (ILD) 的临床,放射和病理特征.

主要方法:

对329名抗MDA5+DM患者的病历进行了回顾性分析.
放射检查和肺部组织病理学 (对17名患者进行).

主要成果:

308名患者 (93.6%) 患有ILD,177人 (53.8%) 患有快速进展的ILD (RPILD).
常见的放射图案包括组织性肺炎 (OP),非特异性间歇性肺炎 (NSIP) 和NSIP+OP.
组织病理学揭示了NSIP和NSIP+OP作为主要模式,放射学和病理学诊断之间的一致性较低 (11.8%).
在RPILD患者中,NSIP+OP的患病率较高,CT上的NSIP的患病率较低.
NSIP+OP或扩散膜损伤 (DAD) 的放射性模式与预后不佳和较高的死亡率有关.

结论:

快速进展的ILD (RPILD) 经常在抗MDA5+DM药物中观察到.
组织性肺炎 (OP) 是主要的放射图案,与NSIP或NSIP+OP组织病理学相关.
NSIP+OP或DAD的放射图案表明,在ILD的抗MDA5+DM患者中,预后不佳.

关键词:

皮肤肌肉炎 (dermatomyositis) 是一种疾病.

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