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@article{Dawes:2023:10.1111/resp.14378,
author = {Dawes, TJW and McCabe, C and Dimopoulos, K and Stewart, I and Bax, S and Harries, C and Samaranayake, CB and Kempny, A and Molyneaux, PL and Seitler, S and Semple, T and Li, W and George, PM and Kouranos, V and Chua, F and Renzoni, EA and Kokosi, M and Jenkins, G and Wells, AU and Wort, SJ and Price, LC},
doi = {10.1111/resp.14378},
journal = {Respirology},
pages = {262--272},
title = {Phosphodiesterase 5 inhibitor treatment and survival in interstitial lung disease pulmonary hypertension: A Bayesian retrospective observational cohort study},
url = {http://dx.doi.org/10.1111/resp.14378},
volume = {28},
year = {2023}
}

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TY  - JOUR
AB  - Background and ObjectivePulmonary hypertension is a life-limiting complication of interstitial lung disease (ILD-PH). We investigated whether treatment with phosphodiesterase 5 inhibitors (PDE5i) in patients with ILD-PH was associated with improved survival.MethodsConsecutive incident patients with ILD-PH and right heart catheterisation, echocardiography and spirometry data were followed from diagnosis to death, transplantation or censoring with all follow-up and survival data modelled by Bayesian methods.ResultsThe diagnoses in 128 patients were idiopathic pulmonary fibrosis (n = 74, 58%), hypersensitivity pneumonitis (n = 17, 13%), non-specific interstitial pneumonia (n = 12, 9%), undifferentiated ILD (n = 8, 6%) and other lung diseases (n = 17, 13%). Final outcomes were death (n = 106, 83%), transplantation (n = 9, 7%) and censoring (n = 13, 10%). Patients treated with PDE5i (n = 50, 39%) had higher mean pulmonary artery pressure (median 38 mm Hg [interquartile range, IQR: 34, 43] vs. 35 mm Hg [IQR: 31, 38], p = 0.07) and percentage predicted forced vital capacity (FVC; median 57% [IQR: 51, 73] vs. 52% [IQR: 45, 66], p=0.08) though differences did not reach significance. Patients treated with PDE5i survived longer than untreated patients (median 2.18 years [95% CI: 1.43, 3.04] vs. 0.94 years [0.69, 1.51], p = 0.003) independent of all other prognostic markers by Bayesian joint-modelling (HR 0.39, 95% CI: 0.23, 0.59, p < 0.001) and propensity-matched analyses (HR 0.38, 95% CI: 0.22, 0.58, p < 0.001). Survival difference with treatment was significantly larger if right ventricular function was normal, rather than abnormal, at presentation (+2.55 years, 95% CI: −0.03, +3.97 vs. +0.98 years, 95% CI: +0.47, +2.00, p = 0.04).ConclusionPDE5i treatment in ILD-PH should be investigated by a prospective randomized trial.
AU  - Dawes,TJW
AU  - McCabe,C
AU  - Dimopoulos,K
AU  - Stewart,I
AU  - Bax,S
AU  - Harries,C
AU  - Samaranayake,CB
AU  - Kempny,A
AU  - Molyneaux,PL
AU  - Seitler,S
AU  - Semple,T
AU  - Li,W
AU  - George,PM
AU  - Kouranos,V
AU  - Chua,F
AU  - Renzoni,EA
AU  - Kokosi,M
AU  - Jenkins,G
AU  - Wells,AU
AU  - Wort,SJ
AU  - Price,LC
DO  - 10.1111/resp.14378
EP  - 272
PY  - 2023///
SN  - 1323-7799
SP  - 262
TI  - Phosphodiesterase 5 inhibitor treatment and survival in interstitial lung disease pulmonary hypertension: A Bayesian retrospective observational cohort study
T2  - Respirology
UR  - http://dx.doi.org/10.1111/resp.14378
UR  - https://www.webofscience.com/api/gateway?GWVersion=2&SrcApp=PARTNER_APP&SrcAuth=LinksAMR&KeyUT=WOS:000861426600001&DestLinkType=FullRecord&DestApp=ALL_WOS&UsrCustomerID=a2bf6146997ec60c407a63945d4e92bb
UR  - https://onlinelibrary.wiley.com/doi/10.1111/resp.14378
UR  - http://hdl.handle.net/10044/1/101622
VL  - 28
ER  -

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