Abstract
BackgroundFollowing 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).MethodsSafety and efficacy of antifibrotic drugs in IPF patients undergoing LTx were investigated in a single-centre retrospective cohort analysis.ResultsA 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.ConclusionsAntifibrotic drugs can probably be safely administered in IPF patients, possibly attenuating disease progression over time, while awaiting LTx.
Highlights
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)
In October 2014, the US Food and Drug Administration (FDA) approved two anti-fibrotic drugs for IPF - pirfenidone and nintedanib - based on the results of large randomized clinical trials (CAPACITY-1, CAPACITY-2 and ASCEND with pirfenidone; TOMORROW, INPULSIS-1 and INPULSIS-2 with nintedanib) demonstrating a reduction in the rate of decline in forced vital capacity (FVC) in mild to moderate IPF [3,4,5]
Post-hoc analysis demonstrated a risk reduction for IPFrelated mortality with pirfenidone compared to placebo (HR 0.32, 95% CI 0.14–0.76, p = 0.006) [6], a same trend which was observed with nintedanib
Summary
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). Idiopathic pulmonary fibrosis (IPF) is a progressive and lethal disease characterized by chronic, fibrosing interstitial pneumonitis of unknown cause, associated with a histopathologic and/or radiologic pattern of usual interstitial pneumonia (UIP) [1]. In October 2014, the US Food and Drug Administration (FDA) approved two anti-fibrotic drugs for IPF - pirfenidone and nintedanib - based on the results of large randomized clinical trials (CAPACITY-1, CAPACITY-2 and ASCEND with pirfenidone; TOMORROW, INPULSIS-1 and INPULSIS-2 with nintedanib) demonstrating a reduction in the rate of decline in forced vital capacity (FVC) in mild to moderate IPF [3,4,5].
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