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Sabrina Hoa¹, Claudie Berger², Nouha Lahmek¹

¹Centre Hospitalier de l'Université de Montréal, Montréal, Québec, Canada.

Arthritis & rheumatology (Hoboken, N.J.)

|November 4, 2024

概括

间歇性肺病 (ILD) 可以在系统性硬化症 (SSc) 的晚期发生. 晚发性ILD与早期的SSc-ILD具有共同的风险因素和进展率,需要持续监测.

科学领域:

类风湿病学类风湿病学
肺部病理学肺部病理学
内部医学内部医学

背景情况:

间歇性肺病 (ILD) 是系统性硬化症 (SSc) 的严重并发症.
通过高分辨率计算机断层扫描 (HRCT) 查ILD,建议SSc患者具有风险因素,包括早期疾病.
关于ILD在SSc过程中晚些时候呈现的特征的数据有限.

研究的目的:

调查系统性硬化症患者晚发性ILD的发病率,风险因素和结果.
将这些特征与早期发病的SSc-ILD进行比较.

主要方法:

来自加拿大硬化皮肤病研究小组队列 (2004-2020年) 没有先前存在的ILD的患者的分析.
从初始非雷诺症的表现开始的疾病持续时间>7年 (晚期) 和<7年 (早期) 的患者之间的ILD发病率和风险因素的比较.
使用卡普兰-梅尔和考克斯回归模型评估ILD进展.

主要成果:

在969名患者中,有199名患者 (21%) 在2.4年的中位随访期内发展出事件性ILD.
与早期的SSc相比,在晚期SSc中ILD的发病率较低 (RR 0.68).
ILD的危险因素包括男性性别,扩散的SSc,肌炎,抗毒素酶I和C反应蛋白的升高. 晚期发病的ILD与关节炎和抗RNA聚合酶III抗体有关.
肺功能严重程度和进展率在晚期和早期发作的SSc-ILD之间是相似的.

结论:

间歇性肺病可以在系统性硬化症的晚期表现出来.
晚发性SSc-ILD的风险因素和进展模式与早发性疾病的风险因素和进展模式相似.
即使在长期SSc患者中,持续监测ILD至关重要,尽管最佳监测频率和方法需要进一步定义.

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