作者
Oksana A Shlobin, Vasilis Kouranos, Scott D Barnett, Esam H Alhamad, Daniel A Culver, Joseph Barney, Francis C Cordova, Eva M Carmona, Mary Beth Scholand, Marlies Wijsenbeek, Sivagini Ganesh, Elyse E Lower, Peter J Engel, John Wort, Laura Price, Athol U Wells, Steven D Nathan, Robert P Baughman
发表日期
2020/5/1
期刊
European Respiratory Journal
卷号
55
期号
5
出版商
European Respiratory Society
简介
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 …
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OA Shlobin, V Kouranos, SD Barnett, EH Alhamad… - European Respiratory Journal, 2020