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

Abstract

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 <300m 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 <300m 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.