系统性硬化症中的近端弱点和肌酸激酶升高:临床相关性,预后和功能影响
Jessica L Fairley1, Dylan Hansen2, Jessica Day3
1The University of Melbourne, Melbourne, Victoria, Australia; St. Vincent's Hospital Melbourne, Melbourne, Victoria, Australia.
Seminars in arthritis and rheumatism
|February 5, 2024
概括
肌肉疾病在全身性硬化症 (SSc) 中很常见. 靠近体弱 (PW) 和肌酸激酶 (CK) 升高表明严重的SSc表型,并显著增加死亡风险.
科学领域:
- 类风湿病学 类风湿病学
- 临床免疫学临床免疫学
- 内部医学 内部医学
背景情况:
- 系统性硬化症 (SSc) 是一种复杂的自身免疫性疾病,其特点是纤维化,血管异常和免疫系统功能障碍.
- 肌肉参与或肌肉病变可能发生在SSc中,但其流行率,临床意义和预后影响需要进一步阐明.
研究的目的:
- 调查SSc患者肌肉疾病临床证据的频率.
- 在SSc.中确定与肌肉疾病相关的临床相关性.
- 确定SSc.肌肉疾病的预后影响.
主要方法:
- 来自澳大利亚硬化皮肤症队列研究的数据分析.
- 参与者根据近端弱点 (PW) 和较高的肌酸激酶 (CK) 水平进行分类.
- 统计分析包括千平方,ANOVA,克鲁斯卡尔-瓦利斯测试和Cox回归用于生存分析.
主要成果:
- 近一半的1786名SSc参与者表现出肌肉疾病 (PW和/或升高的CK) 的临床迹象.
- 同时的PW和升高的CK与严重的SSc表型相关,包括分散的皮肤SSc,肌摩擦擦,肌炎和数字.
- 带有或没有升高CK的PW与增加的多发病率,心肺疾病和显著更高的死亡率有关.
- PW也与身体功能受损有关,由减少6分钟步行距离和更高的HAQ-DI得分证明.
结论:
- 肌肉病的临床表现在全身性硬化症中非常普遍.
- 即使没有明确的SSc-肌肉病诊断,近端弱度和/或肌肉酸酶升高的存在,也确定了具有显著临床关联和更差预后的患者.
- 早期发现和管理肌肉参与SSc对于改善患者的治疗结果至关重要.
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