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

The European Respiratory Journal
Oksana A ShlobinRobert P Baughman

Abstract

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. 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. 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 (...Continue Reading

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Citations

Aug 3, 2020·Current Opinion in Pulmonary Medicine·Debabrata Bandyopadhyay, Marc Humbert
Aug 11, 2020·Seminars in Respiratory and Critical Care Medicine·Marloes P HuitemaMarco C Post
May 16, 2020·The European Respiratory Journal·Daniel VisJason Weatherald
Dec 2, 2020·Journal of Clinical Medicine·Esam H AlhamadWaleed M Alharbi
Jan 29, 2021·Frontiers in Medicine·Naureen Narula, Michael Iannuzzi
Mar 3, 2021·European Journal of Haematology·Cassondra Cramer-BourElizabeth S Klings
May 3, 2021·Heart, Lung & Circulation·Harold MathijssenMarco C Post
Jun 7, 2021·Pulmonary Therapy·Andrea S MelaniElena Bargagli
Jun 16, 2021·Current Opinion in Pulmonary Medicine·Chinthaka B SamaranayakeLaura C Price

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