The effect of antifibrotics on the progression of pulmonary hypertension in patients with interstitial lung disease listed for lung transplantation

Purpose: Antifibrotics (AF) attenuate the progression of fibrotic interstitial lung disease (ILD), but it is unknown if these drugs affect the progression of pulmonary hypertension (PH) in patients with ILD. We explored whether use of AF therapy was associated with a lower rate of change of mean pulmonary artery pressure (mPAP) in patients with ILD listed for lung transplantation (LTx). Methods: All LTx recipients at Inova Fairfax Hospital from 2012 to 2023 with a pre-LTx diagnosis of ILD qualified for the analysis. Demographic data, mPAP at the time of LTx listing, intra-operative mPAP during lung transplantation, use of AF and PH therapy, spirometry, and 6-minute walk test data were collated. 153 patients were included the analysis. The mPAP rate of change (mmHg/month) was compared between patients based on the administration of AF in the pre-LTx period. Results: The mPAP average rate of change was 0.9 mmHg/month for the AF group and 0.7 mmHg/month for the no AF group (p = 0.504). On multivariate analysis, age and the use of PH therapy were associated with mPAP rate of change. After accounting for these factors, AF usage was not associated with mPAP rate of change (p = 0.194). Additionally, AF usage was not found to augment the effects of PH therapy on mPAP change. Age was associated with a faster rate of change of mPAP, and PH therapy was associated with slower rate of change in mPAP. Conclusion: AF therapy was not associated with a lower rate of change in mPAP in patients with ILD listed for LTx.