ANN RHEUM DIS:早期诊断的系统性硬化症相关性肺动脉高压进展的相关因素

2017-10-25 xiangting MedSci原创

40%以上的早期诊断SSc-PAH患者在短期内有疾病进展,男性、PAH诊断时的功能状况和肺功能与进展相关。

肺动脉高压(PAH)是系统性硬化症(SSc)的严重并发症。这项纵向研究旨在确定DETECT队列中与患早期PAH的SSc患者(SSc-PAH)不良结局相关的因素。

对DETECT队列中的SSc-PAH患者观察3年。使用单变量logistic回归来分析横断面变量与疾病进展之间的关联(疾病进展定义为出现以下任何事件:WHO功能分类恶化,PAH联合治疗,住院或死亡)。

57例PAH患者(中位观察时间12.6个月)中,25例(43.9%)有疾病进展。以下因素(OR(95%CI))与疾病进展相关:男性(4.1(1.2-14.1)),用力肺活量占预测值百分比%/一氧化碳弥散能力(DLCO)占预测值百分比%升高(3.6(1.2-10.7)),Borg呼吸困难指数升高(1.7(1.1-2.6))和DLCO占预测值百分比%降低(非线性关系)。

40%以上的早期诊断SSc-PAH患者在短期内有疾病进展,男性、PAH诊断时的功能状况和肺功能与进展相关。这表明即使是轻度的PAH也应被认为是SSc的高危并发症。

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