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We investigated whether treatment with phosphodiesterase 5 inhibitors\u00a0(PDE5i) in patients with ILD\u2010PH was associated with improved survival.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Consecutive incident patients with ILD\u2010PH and right heart catheterisation, echocardiography and spirometry data were followed from diagnosis to death, transplantation or censoring with all follow\u2010up and survival data modelled by Bayesian methods.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The diagnoses in 128 patients were idiopathic pulmonary fibrosis (<jats:italic>n<\/jats:italic>\u00a0=\u00a074, 58%), hypersensitivity pneumonitis (<jats:italic>n<\/jats:italic>\u00a0=\u00a017, 13%), non\u2010specific interstitial pneumonia (<jats:italic>n<\/jats:italic>\u00a0=\u00a012, 9%), undifferentiated ILD (<jats:italic>n<\/jats:italic>\u00a0=\u00a08, 6%) and other lung diseases (<jats:italic>n<\/jats:italic>\u00a0=\u00a017, 13%). Final outcomes were death (<jats:italic>n<\/jats:italic>\u00a0=\u00a0106, 83%), transplantation (<jats:italic>n<\/jats:italic>\u00a0=\u00a09, 7%) and censoring (<jats:italic>n<\/jats:italic>\u00a0=\u00a013, 10%). Patients treated with PDE5i (<jats:italic>n<\/jats:italic>\u00a0=\u00a050, 39%) had higher mean pulmonary artery pressure (median 38\u2009mm Hg [interquartile range, IQR: 34, 43] vs. 35\u2009mm Hg [IQR: 31, 38], <jats:italic>p\u2009=\u2009<\/jats:italic>0.07)\u00a0and percentage predicted forced vital capacity (FVC; median 57% [IQR: 51, 73] vs. 52% [IQR: 45, 66], <jats:italic>p=<\/jats:italic>0.08) though differences did not reach significance. Patients treated with PDE5i survived longer than untreated patients (median 2.18\u2009years [95% CI: 1.43, 3.04] vs. 0.94\u2009years [0.69, 1.51], <jats:italic>p\u00a0=<\/jats:italic>\u00a00.003) independent of all other prognostic markers by Bayesian joint\u2010modelling (HR 0.39, 95% CI: 0.23, 0.59, <jats:italic>p<\/jats:italic>\u2009&lt;\u20090.001) and propensity\u2010matched analyses (HR 0.38, 95% CI: 0.22, 0.58, <jats:italic>p<\/jats:italic>\u2009&lt;\u20090.001). Survival difference with treatment was significantly larger if right ventricular function was normal, rather than abnormal, at presentation (+2.55\u2009years, 95% CI: \u22120.03, +3.97 vs. +0.98\u2009years, 95% CI: +0.47, +2.00, <jats:italic>p<\/jats:italic>\u00a0=\u00a00.04).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>PDE5i treatment in ILD\u2010PH should be investigated by a prospective randomized trial.<\/jats:p><\/jats:sec>","DOI":"10.1111\/resp.14378","type":"journal-article","created":{"date-parts":[[2022,9,29]],"date-time":"2022-09-29T09:38:43Z","timestamp":1664444323000},"page":"262-272","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":52,"title":["Phosphodiesterase 5 inhibitor treatment and survival in interstitial lung disease pulmonary hypertension: A Bayesian retrospective observational cohort study"],"prefix":"10.1111","volume":"28","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7871-524X","authenticated-orcid":false,"given":"Timothy J. 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