Physiological predictors of survival in patients with sarcoidosis-associated pulmonary hypertension: results from an international registry

Autor: Eva M. Carmona, Laura C. Price, Marlies S. Wijsenbeek, Athol U. Wells, John Wort, Vasilis Kouranos, Joseph Barney, Elyse E. Lower, Oksana A. Shlobin, Peter J. Engel, Esam H. Alhamad, Steven D. Nathan, Scott D. Barnett, Daniel A. Culver, Robert P. Baughman, Sivagini Ganesh, Mary Beth Scholand, Francis Cordova
Přispěvatelé: Pulmonary Medicine
Jazyk: angličtina
Rok vydání: 2020
Předmět:
Zdroj: European Respiratory Journal, 55(5):1901747. European Respiratory Society
ISSN: 0903-1936
Popis: IntroductionSarcoidosis-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.MethodsReSAPH 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.ResultsData 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) (1)/forced vital capacity (FVC) ratio were identified as independent risk factors for reduced transplant-free survival in the precapillary cohort.ConclusionReduced DLCO (1/FVC ratio was identified as an independent risk factor for worsened outcomes.
Databáze: OpenAIRE