Introduction and objectives Lung transplantation provides a significant survival benefit to patients with advanced idiopathic pulmonary fibrosis (IPF). However, at this time, the transplant community is unable to meet the requirements on it services due to donor organ shortages. This results in an increased length of time spent on the waiting list and an increased risk of death prior to transplantation.
Pirfenidone has been reported to reduce the rate of disease progression in patients with IPF. It may therefore prolong the length of time that patients are able to spend on the transplant waiting list. We report the outcomes of three patients with progressive IPF who were successfully bridged to transplantation with Pirfenidone.
Methods We retrospectively reviewed the medical records of all patients who had undergone lung transplantation for IPF from 2012–14 at our institution. Three patients who had been prescribed Pirfenidone prior to transplantation were identified. Each patient continued Pirfenidone until the day of transplantation. Patient demographics, lung function and post transplant data were collated.
Results Prior to the commencement of Pirfenidone the mean decline in forced vital capacity (FVC) was 52.2ml per month. Following Pirfenidone therapy, the mean decline in FVC was 29.2ml per month. The mean length of time from commencing Pirfenidone to transplantation was 419 days (range 190–768 days). The mean length of time spent on the transplant waiting list was 144 days (range 35–271 days).
With a mean follow up of 1.45 years, no episodes of acute or chronic rejection have occurred. Post-transplant survival is 100%. No adjustment in immunosuppressant induction or post-transplant therapy was necessitated. In the post-transplant period, Pirfenidone therapy was not linked to any adverse events.
Conclusion Pirfenidone has been reported to reduce disease progression in IPF. However, despite this, lung transplantation remains necessary in the management of this condition. For patients with IPF, in whom the transplant window is short, Pirfenidone may allow for valuable added time on the lung transplant waiting list.
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