{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,24]],"date-time":"2026-09-24T16:22:20Z","timestamp":1790266940788,"version":"4.1.0"},"reference-count":39,"publisher":"European Respiratory Society (ERS)","issue":"5","license":[{"start":{"date-parts":[[2020,3,5]],"date-time":"2020-03-05T00:00:00Z","timestamp":1583366400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/www.ersjournals.com\/user-licence"}],"content-domain":{"domain":["publications.ersnet.org"],"crossmark-restriction":true},"short-container-title":["Eur Respir J"],"accepted":{"date-parts":[[2020,2,11]]},"published-print":{"date-parts":[[2020,5]]},"abstract":"<jats:sec><jats:title>Introduction<\/jats:title><jats:p>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.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>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.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Data from 215 patients followed for a mean\u00b1<jats:sc>sd<\/jats:sc>2.5\u00b11.9\u2005years were available for analysis. In the 159 precapillary patients, the Kaplan\u2013Meier-adjusted 1-, 3- and 5-year transplant-free survival was 89.2%, 71.7% and 62.0%, respectively. Kaplan\u2013Meier-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 (<jats:italic>D<\/jats:italic><jats:sub>LCO<\/jats:sub>) (&lt;35% predicted) and 6MWD &lt;300\u2005m in the precapillary cohort had significantly worse transplant-free survival. Reduced 6MWD and preserved forced expiratory volume (FEV<jats:sub>1<\/jats:sub>)\/forced vital capacity (FVC) ratio were identified as independent risk factors for reduced transplant-free survival in the precapillary cohort.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Reduced<jats:italic>D<\/jats:italic><jats:sub>LCO<\/jats:sub>(&lt;35% pred) and 6MWD (&lt;300\u2005m) at the time of registry enrolment were associated with reduced transplant-free survival in the overall precapillary cohort. Preserved FEV<jats:sub>1<\/jats:sub>\/FVC ratio was identified as an independent risk factor for worsened outcomes.<\/jats:p><\/jats:sec>","DOI":"10.1183\/13993003.01747-2019","type":"journal-article","created":{"date-parts":[[2020,3,5]],"date-time":"2020-03-05T13:25:14Z","timestamp":1583414714000},"page":"1901747","update-policy":"https:\/\/doi.org\/10.1183\/ers-crossmark-policy","source":"Crossref","is-referenced-by-count":90,"title":["Physiological predictors of survival in patients with sarcoidosis-associated pulmonary hypertension: results from an international registry"],"prefix":"10.1183","volume":"55","author":[{"given":"Oksana A.","family":"Shlobin","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vasilis","family":"Kouranos","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Scott 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