％0刊杂志百分之一什叶岛，oksana a.％akouranos，vasilis％a barnett，斯科特d.％a阿尔马德，eSam H.％a Culver，Daniel A.％A Barney，Joseph％的Cordova，弗朗西斯C.％一个Carmona，Eva M.％A Scholand，Mary Beth％A Wijsenbeek，Marlies％A Ganesh，Sivagini％较低，Elyse E.％Angel，彼得J.％的麦汁，John％的价格，Laura％的井，athol u.％a Nathan，Steven D.％A Baughman，罗伯特P.％患者生存患者生存的生理学预测因素 - 相关的肺动脉高压：国际寄存器％D 2020％R 10.17.01747-2019％J欧洲的结果呼吸期轴颈％P 1901747％V 55％N 5％X引言结节病相关肺动脉高压（Saph）与单中心研究中的存活率降低有关。建立了长期随访的Saph（退休表）的国际登记处，以丰富我们对这种结节病的并并发症的了解。该分析旨在阐明与Saph患者的无流体存活相关的因素。由于登记登记时间以来，含水患者含有前瞻性收集的Saph患者的结果。分析的信息包括右心导管数据，肺功能测试，胸部射线照相，诈骗阶段和6分钟步行距离（6MWD）。Cox回归模型用于识别无自由移植存活的独立预测因子。结果来自215名患者的数据，然后进行平均±1.9岁以进行分析。在159名前一种患者中，Kaplan-Meier调整的1-，3-和5年的无移植存活分别为89.2％，71.7％和62.0％。事件组的Kaplan-Meier调整后的1-，3-和5年的无移植存活率分别为83.5％，分别为70.3％和58.3％，分别为94.7％，72.2％和66.3％。用于一氧化碳（DLCO）的肺部扩散能力降低的患者（预测的35％）和6MWD <300米在预先群组中的移植存活率显着差。 Reduced 6MWD and preserved forced expiratory volume (FEV1)/forced vital capacity (FVC) ratio were identified as independent risk factors for reduced transplant-free survival in the precapillary cohort.Conclusion Reduced DLCO (<35% pred) and 6MWD (<300 m) at the time of registry enrolment were associated with reduced transplant-free survival in the overall precapillary cohort. Preserved FEV1/FVC ratio was identified as an independent risk factor for worsened outcomes.Decreased 6-min walk distance and reduced diffusion capacity are associated with decreased survival in patients with sarcoidosis-associated pulmonary hypertension http://bit.ly/2UQfWJh %U //www.qdcxjkg.com/content/erj/55/5/1901747.full.pdf