RT Journal Article
SR Electronic
T1 Physiological Predictors of Survival in Patients with Sarcoidosis Associated Pulmonary Hypertension: Results from an International Registry
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP 1901747
DO 10.1183/13993003.01747-2019
A1 Oksana A. Shlobin
A1 Vasilis Kouranos
A1 Scott D. Barnett
A1 Esam H. Alhamad
A1 Daniel A. Culver
A1 Joseph Barney
A1 Francis C. Cordova
A1 Eva M. Carmona
A1 Mary Beth Scholand
A1 Marlies Wijsenbeek
A1 Sivagini Ganesh
A1 Elyse E. Lower
A1 Peter J. Engel
A1 John Wort
A1 Laura Price
A1 Athol U. Wells
A1 Steven D. Nathan
A1 Robert P. Baughman
YR 2020
UL http://erj.ersjournals.com/content/early/2020/03/17/13993003.01747-2019.abstract
AB Introduction Sarcoidosis associated pulmonary hypertension (SAPH) is associated with reduced survival in single center studies. An 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 enrollment. Information analyzed includes right heart catheterization data, pulmonary function testing, chest x-ray Scadding stage, six minute walk distance (6MWD) among others. Cox regression models were used to identify independent predictors of transplant-free survival.Results Data from a total of 215 patients followed for a mean of 2.5±1.9 years were available for analysis. In the 159 pre-capillary patients, the KM adjusted 1, 3 and 5 year transplant free survival was 89.2%, 71.7% and 62.0%, respectively. In the incident and prevalent groups, KM adjusted 1, 3 and 5 year transplant free survival was 83.5%, 70.3% and 58.3% and 94.7%, 72.2%, and 66.3% respectively. Patients with reduced DLCO (<35% predicted) and 6MWD <300 m in the pre-capillary cohort had significantly worse transplant-free survival. Reduced 6MWD and preserved FEV1/FVC ratio were identified as independent risk factors for reduced transplant-free survival in the pre-capillary cohort.Conclusion Reduced diffusion capacity (<35% of predicted) and 6MWD <300 m at the time of registry enrollment were associated with reduced transplant-free survival in the overall precapillary cohort. Preserved FEV1/FVC ratio was also identified as an independent risk factor for worsened outcomes.FootnotesThis manuscript has recently been accepted for publication in the European Respiratory Journal. It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJ online. Please open or download the PDF to view this article.Conflict of interest: Dr. Shlobin reports personal fees from United Therapeutics, personal fees from Bayer, personal fees from Johnson & Johnson, outside the submitted work.Conflict of interest: Dr. Kouranos has nothing to disclose.Conflict of interest: Dr. Barnett has nothing to disclose.Conflict of interest: Dr. Alhamad has nothing to disclose.Conflict of interest: Dr. Culver reports other from Gilead, during the conduct of the study; personal fees from Johnson and Johnson, outside the submitted work.Conflict of interest: Dr. Barney has nothing to disclose.Conflict of interest: Dr. Cordova has nothing to disclose.Conflict of interest: Dr. Carmona reports other from Gilead, during the conduct of the study; personal fees from consulting to develop education materials, CHEST, personal fees from editor for Elsevier, outside the submitted work.Conflict of interest: Dr. Scholand reports other from Boehringer Ingelheim, other from Genentech, other from Fibrogen, other from Global Blood Therapeutics, outside the submitted work; In addition, Dr. Scholand has a patent Apparatus, Compositions and Methods for Assessment of Chronic Obstructive Pulmonary Disease Progression among Rapid and Slow Decline Conditions issued.Conflict of interest: Dr. wijsenbeek has nothing to disclose.Conflict of interest: Dr. Ganesh has nothing to disclose.Conflict of interest: Dr. Lower reports grants from Gilead, grants from Bayer, grants from Genentech, during the conduct of the study.Conflict of interest: Dr. Engel has nothing to disclose.Conflict of interest: Dr. Wort reports grants and personal fees from Actelion Pharmaceuticals, grants and personal fees from Bayer Pharmaceuticals, personal fees from MSD, outside the submitted work.Conflict of interest: Dr. Price reports personal fees and other from Actelion Johnson&Johnson, during the conduct of the study.Conflict of interest: Dr. Wells reports personal fees from Boeringher Ingelheim, personal fees from Roche, personal fees from Intermune, personal fees from Bayer, outside the submitted work.Conflict of interest: Dr. Nathan reports personal fees from United Therapeutics, personal fees from Bayer Pharmaceuticals, personal fees from Bellerophon, personal fees from Actelion, during the conduct of the study.Conflict of interest: Dr. Baughman reports grants from Gilead, grants and personal fees from Actelion, grants from Bayer, grants and personal fees from Mallinckrodt, grants from Genentech, grants from Foundation for sarcoidosis research, grants from National institutes of health, during the conduct of the study.