由此可见TY -的T1 -生理的预测因素l in patients with sarcoidosis-associated pulmonary hypertension: results from an international registry JF - European Respiratory Journal JO - Eur Respir J DO - 10.1183/13993003.01747-2019 VL - 55 IS - 5 SP - 1901747 AU - Shlobin, Oksana A. AU - Kouranos, Vasilis AU - Barnett, Scott D. AU - Alhamad, Esam H. AU - Culver, Daniel A. AU - Barney, Joseph AU - Cordova, Francis C. AU - Carmona, Eva M. AU - Scholand, Mary Beth AU - Wijsenbeek, Marlies AU - Ganesh, Sivagini AU - Lower, Elyse E. AU - Engel, Peter J. AU - Wort, John AU - Price, Laura AU - Wells, Athol U. AU - Nathan, Steven D. AU - Baughman, Robert P. Y1 - 2020/05/01 UR - //www.qdcxjkg.com/content/55/5/1901747.abstract N2 - Introduction Sarcoidosis-associated pulmonary hypertension (SAPH) is associated with reduced survival in single-centre studies. The international Registry for SAPH (ReSAPH) with long-term follow-up was established to enrich our knowledge of this complication of sarcoidosis. This analysis aims to elucidate factors associated with reduced transplant-free survival in SAPH patients.Methods ReSAPH contains prospectively collected outcomes of SAPH patients since the time of registry enrolment. Information analysed includes right heart catheterisation data, pulmonary function testing, chest radiography, Scadding stage and 6-min walk distance (6MWD), among others. Cox regression models were used to identify independent predictors of transplant-free survival.Results Data from 215 patients followed for a mean±sd 2.5±1.9 years were available for analysis. In the 159 precapillary patients, the Kaplan–Meier-adjusted 1-, 3- and 5-year transplant-free survival was 89.2%, 71.7% and 62.0%, respectively. 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 -