Safety and efficacy of bridging to lung transplantation with antifibrotic drugs in idiopathic pulmonary fibrosis: a case series

Isabelle Delanote, Wim A Wuyts, Jonas Yserbyt, Eric K Verbeken, Geert M Verleden, Robin Vos, Isabelle Delanote, Wim A Wuyts, Jonas Yserbyt, Eric K Verbeken, Geert M Verleden, Robin Vos

Abstract

Background: Following recent approval of pirfenidone and nintedanib for idiopathic pulmonary fibrosis (IPF), questions arise about the use of these antifibrotics in patients awaiting lung transplantation (LTx).

Methods: Safety and efficacy of antifibrotic drugs in IPF patients undergoing LTx were investigated in a single-centre retrospective cohort analysis.

Results: A total of nine patients, receiving antifibrotic therapy for 419 ± 315 days until subsequent LTx, were included. No major side effects were noted. Significant weight loss occurred during antifibrotic treatment (p = 0.0062). FVC tended to stabilize after 12 weeks of treatment in most patients. A moderate decline in FVC, TLC and DLCO was noted during the whole pretransplant time period of antifibrotic therapy. Functional exercise capacity and lung allocation score remained unchanged. No post-operative thoracic wound healing problems, nor severe early anastomotic airway complications were attributable to prior antifibrotic treatment. None of the patients developed chronic lung allograft dysfunction after a median follow-up of 19.8 (11.2-26.5) months; and post-transplant survival was 100% after 1 year and 80% after 2 years.

Conclusions: Antifibrotic drugs can probably be safely administered in IPF patients, possibly attenuating disease progression over time, while awaiting LTx.

Keywords: Antifibrotics; IPF; Lung transplantation; Nintedanib; Pirfenidone; Safety.

Figures

Fig. 1
Fig. 1
Forced Vital Capacity in IPF patients with at least 6 months antifibrotic therapy before transplantation. Forced Vital Capacity (FVC) (%predicted) is given at the start of antifibrotic therapy (start), 3 months before and respectively 3, 6, 9 and 12 months (mo) after start. Dotted lines connect values in patients (n = 6/9) with consecutive measurements at different time points; p-values (Wilcoxon signed rank test) above each time point are given compared to start; or compared another time point (time-frame indicated by full line)
Fig. 2
Fig. 2
Pretransplant evolution of pulmonary function and functional exercise capacity following treatment with antifibrotic drugs. Forced Vital Capacity (FVC) (a), Total Lung Capacity (TLC) (b), Diffusion capacity (DLCO) (c) (all in (%predicted) and 6 min walk test (6MWT, meter) (d) at start of antifibrotic therapy (start) and at the moment of lung transplantation (LTx) in the included IPF patients. Dotted lines connect values in patients (n = 6/9) with a consecutive measurement at six months and just before transplantation; p-values (Wilcoxon signed rank test) are given for patients that had consecutive measurements

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