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

European Respiratory Journal Apr 01, 2020

Shlobin OA, Kouranos V, Barnett SD, et al. - To define factors related to decreased transplant-free survival in sarcoidosis associated pulmonary hypertension (SAPH) patients, researchers undertook this investigation using ReSAPH (an international registry for SAPH) that comprises prospectively gathered results of SAPH patients since the time of registry enrollment. They studied data from 215 patients who were followed for a mean of 2.5 ± 1.9 years. The KM adjusted 1, 3 and 5 year transplant free survival was estimated to be 89.2%, 71.7% and 62.0%, respectively, in the 159 pre-capillary patients. According to the findings, decreased diffusion capacity (<35% of predicted) as well as six minute walk distance < 300 m at the time of registry enrollment were found to be related to decreased transplant-free survival in the entire precapillary cohort. Preserved FEV1/FVC ratio behaved as an independent risk factor for worsened results.

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