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 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 analysed includes right heart catheterisation data, pulmonary function testing, chest x-ray Scadding stage, 6 min 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 1, 3 and 5 year transplant free survival was 98.2.1%, 78.2% and 71.2%, respectively. Incident group 83.5%, 70.3% and 58.3% and prevalent group 94.7%, 72.2%, 66.3% 1,3 and 5 year survival, respectively. Patients with reduced DLCO and 6MWD Conclusion Reduced diffusion capacity and 6MWD