合并间质性肺炎和肺动脉高压的系统性硬化症患者比仅合并肺动脉高压者预后差



              

                    作者:Michelfelder M

   翻译:北医三院沈涛(fengguxuan@163.com

                   发布:高爽     审核:刘佩玲

摘要


已发布的证据提示,间质性肺病(ILD)对系统性硬化病人和肺动脉高压(PAH) 的死亡率有影响,但是否通过血流动力学、运动能力、自身抗体谱和肺功能表现出来暂不明确。在这个部分回顾性研究中,27SSc-PAH患者SSc-PAH合并ILD患者进行生存率、肺功能、血流动力学、运动能力及实验室指标的比较。SSc-PAH-ILD患者明显比SSc-PAH患者预后差(1310年生存率分别为865454%969282%, p = 0.013)。与单纯SSc-PAH患者相比,SSc-PAH-ILD患者表现为诊断PAH时、1年和2年后1s用力呼气量(FEV1)的下降(p = 0.002),两组别并没有PAH进程的显著恶化。在PAH诊断时,ILD-PAH组患者肺一氧化碳弥散量(DLCO)更低。并存的ILD与更差的运动能力、不同的FEV1/FVC比率、更高的WHO功能分级、更低的血流动力不相关。高滴度的血管紧张素和内皮素受体抗体预示着所有SSc-PAH患者的预后,但不能依此区分PAH患者是否合并有ILD。我们的研究证实了ILD对于SSc-PAH患者死亡率的影响。肺功能指标可以被运用于区分患者是否合并ILD。更高的死亡率并不能用血流动力学的差异、运动能力或者自身抗体水平解释,其机制需要进一步的研究。


附原文



Abstract: Published data suggest thatcoexisting interstitial lung disease (ILD) has an impact on mortality inpatients with systemic sclerosis (SSc) and pulmonary arterial hypertension(PAH), but there is scarce knowledge if this is reflected by hemodynamics,exercise capacity, autoantibody profile, or pulmonary function. In thispartially retrospective study, 27 SSc-PAH patients were compared to 24 SSc-PAHpatients with coexisting ILD respecting to survival, pulmonary function,hemodynamics, exercise capacity, and laboratory parameters. Survival wassignificantly worse in SSc-PAH-ILD patients than in SSc patients with isolatedPAH (1, 5, and 10-year survival rates 86, 54, and 54% versus 96, 92, and 82%, p= 0.013). Compared to isolated SSc-PAH patients, patients with SSc-PAH-ILDrevealed lower forced expiratory volume after 1 s (FEV1) values at the time ofPAH diagnosis as well as 1 and 2 years later (p = 0.002) without significantdecrease in the PAH course in both groups. At PAH diagnosis, diffusion capacityfor carbon monoxide (DLCO) values were lower in the ILD-PAH group. CoexistingILD was not associated with lower exercise capacity, different FEV1/forcedvital capacity (FVC) ratio, higher WHO functional class, or reducedhemodynamics. Higher levels of antibodies against angiotensin and endothelinreceptors predict mortality in all SSc-PAH patients but could not differentiatebetween PAH patients with and without ILD. Our study confirmed an impact of ILDon mortality in SSc-PAH patients. Pulmonary function parameters can be used todistinguish PAH from PAH-ILD. The higher mortality rate cannot be explained bydifferences in hemodynamics, exercise capacity, or autoantibody levels.Mechanisms of mortality remain to be studied.



引自



Michelfelder M,Becker M2, Riedlinger A, et al. Interstitial lung disease increases mortalityin systemic sclerosis patients with pulmonary arterial hypertension withoutaffecting hemodynamics and exercise capacity. Clin Rheumatol. 2016, [Epub aheadof print].







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