结节病相关性肺动脉高压患者生存的生理预测因子:结果从一个国际注册JF -欧洲呼吸杂志》乔和J - 10.1183/13993003.01747 -2019欧元六世- 55 - 5 SP - 1901747 AU Shlobin Oksana a . AU - Kouranos Vasilis AU -巴内特,斯科特·d . AU - Alhamad西法h . AU -斑鸠,丹尼尔·a . AU -巴尼,约瑟夫•科尔多瓦AU -弗朗西斯·c . AU -卡伊娃m . AU - Scholand,玛丽•贝思盟——Wijsenbeek土地肥沃的非盟,Ganesh Sivagini AU -低,艾丽丝e . AU -恩格尔,Peter J . AU -麦芽汁,约翰盟——价格,劳拉AU -井,在单中心研究中，结节病相关的肺动脉高压(SAPH)与存活率降低有关。我们建立了具有长期随访的SAPH国际登记(ReSAPH)，以丰富我们对这种结节病并发症的认识。该分析旨在阐明与SAPH患者无移植生存期减少相关的因素。方法ReSAPH包含自注册登记之时起收集的SAPH患者的前瞻性结果。分析的信息包括右心导管数据、肺功能测试、胸片、Scadding分期和6分钟步行距离(6MWD)等。Cox回归模型用于确定无移植生存的独立预测因子。结果分析了215例随访患者的数据，平均±sd(2.5±1.9)年。在159例毛细血管前患者中，kaplan - meier调整后的1、3和5年无移植生存率分别为89.2%、71.7%和62.0%。 Kaplan–Meier-adjusted 1-, 3- and 5-year transplant-free survival in the incident group was 83.5%, 70.3% and 58.3%, respectively, and in the prevalent group was 94.7%, 72.2% and 66.3%, respectively. Patients with reduced diffusing capacity of the lung for carbon monoxide (DLCO) (<35% predicted) and 6MWD <300 m in the precapillary cohort had significantly worse transplant-free survival. 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 ER -