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

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 ( D _LCO ) (<35% predicted) and 6MWD <300 m in the precapillary cohort had significantly worse transplant-free survival. Reduced 6MWD and preserved forced expiratory volume (FEV ₁ )/forced vital capacity (FVC) ratio were identified as independent risk factors for reduced transplant-free survival in the precapillary cohort.
Conclusion: Reduced D _LCO (<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 FEV ₁ /FVC ratio was identified as an independent risk factor for worsened outcomes.
Competing Interests: Conflict of interest: O.A. Shlobin reports personal fees for consultancy from United Therapeutics, Bayer and Johnson & Johnson, outside the submitted work. Conflict of interest: V. Kouranos has nothing to disclose. Conflict of interest: S.D. Barnett has nothing to disclose. Conflict of interest: E.H. Alhamad has nothing to disclose. Conflict of interest: D.A. Culver reports institutional funding for the registry from Gilead, during the conduct of the study; personal fees for advisory board work from Johnson and Johnson, outside the submitted work. Conflict of interest: J. Barney has nothing to disclose. Conflict of interest: F.C. Cordova has nothing to disclose. Conflict of interest: E.M. Carmona reports financial support for the clinical trial from Gilead, during the conduct of the study; personal fees for consultancy and developing education materials from CHEST, personal fees for editorial work from Elsevier, outside the submitted work. Conflict of interest: M.B. Scholand has performed advisory board work and been investigator in clinical trials for Boehringer Ingelheim and Genentech, and been investigator in clinical trials for Fibrogen and Global Blood Therapeutics, outside the submitted work; and 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: M. Wijsenbeek has nothing to disclose. Conflict of interest: S. Ganesh has nothing to disclose. Conflict of interest: E.E. Lower reports grants from Gilead, Bayer and Genentech, during the conduct of the study. Conflict of interest: P.J. Engel has nothing to disclose. Conflict of interest: J. Wort reports grants and personal fees from Actelion Pharmaceuticals and Bayer Pharmaceuticals, personal fees from MSD, outside the submitted work. Conflict of interest: L. Price reports personal fees and education support from Actelion and Johnson & Johnson, during the conduct of the study. Conflict of interest: A.U. Wells reports personal fees from Boehringer Ingelheim, Roche, Intermune and Bayer, outside the submitted work. Conflict of interest: S.D. Nathan reports personal fees for consultancy from United Therapeutics, Bayer Pharmaceuticals, Bellerophon and Actelion, during the conduct of the study. Conflict of interest: R.P. Baughman reports grants from Gilead, Bayer, Genentech, Foundation for Sarcoidosis Research and National Institutes of Health, grants and personal fees for consultancy from Actelion, grants and personal fees for consultancy and lectures from Mallinckrodt, during the conduct of the study.
(Copyright ©ERS 2020.)