在ASyS和SjD重叠的患者中ILD的临床和分子特征:一项追溯观察性研究
Yulu Qiu1, Chengyin Lv1, Yue Wang1
1Department of Rheumatology, The First Affiliated Hospital of Nanjing Medical University, Nanjing, Jiangsu, China.
Rheumatology (Oxford, England)
|October 29, 2025
概括
抗合成酶综合征 (ASyS) 和Sjögren综合征的重叠
科学领域:
- 类风湿病学 类风湿病学
- 肺部病理学 肺部病理学
- 免疫学 免疫学 免疫学
背景情况:
- 间歇性肺病 (ILD) 是抗合成酶综合征 (ASyS) 和原发性肖格伦病 (pSjD) 的常见并发症.
- 患有重叠ASyS和pSjD (ASyS-SjD) 的患者的临床和分子特征尚未确定.
- 了解ASyS-SjD对于将其作为一个独立的实体与偶然的共同发生区分至关重要.
研究的目的:
- 描述具有ILD的ASyS-SjD患者的临床,血清学,放射学和转录学特征.
- 要确定ASyS-SjD是否代表一个不同的疾病实体.
- 将ASyS-SjD中的ILD表型与ASyS和psjD进行比较.
主要方法:
- 对131名ASyS,78名pSjD和42名ASyS-SjD患者进行了回顾性分析.
- 临床特征,实验室发现,肺功能和高分辨率计算机断层扫描 (HRCT) 模式的比较.
- 来自ASyS,pSjD,ASyS-SjD患者和对照患者的外周血液单核细胞的RNA测序.
主要成果:
- 与pSjD相比,ASyS-SjD患者呈现出较早的ILD发病率和更频繁的快速进展ILD (RP-ILD).
- 肺功能在ASyS-SjD患者中比pSjD患者更差.
- 在ASyS-SjD中,HRCT主要显示非特异性间歇性肺炎 (NSIP),类似于ASyS. 转录学揭示了与ASyS共享的炎症以及ASyS-SjD中独特的益菌性信号传递.
结论:
- ASyS-SjD表现出一种类似ASyS的临床表型,具有额外的益菌性特征.
- 这表明ASyS-SjD可能是一个重叠的亚型而不是巧合.
- ASyS-SjD中的ILD似乎比SjD更具侵略性,但不比ASyS严重得多.
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