Lung transplant candidates with idiopathic pulmonary fibrosis and long-term pirfenidone therapy: Treatment feasibility influences waitlist survival |
| |
Authors: | Shin Tanaka Kentaroh Miyoshi Hisao Higo Takeshi Kurosaki Shinji Otani Seiichiro Sugimoto Masaomi Yamane Katsuyuki Kiura Shinichi Toyooka Takahiro Oto |
| |
Affiliation: | 1. Department of Thoracic Surgery, Okayama University Hospital, 2-5-1, Shikata-cho, Kita-ku, Okayama 700-8558, Japan;2. Department of Thoracic Surgery, Okayama Medical Center, 1711-1 Tamasu, Kita-ku, Okayama 701-1192, Japan;3. Respiratory Medicine, Okayama University Hospital, 2-5-1 Shikata-cho, Kita-ku, Okayama 700-8558, Japan;4. Organ Transplant Center, Okayama University Hospital, 2-5-1 Shikata-cho, Kita-ku, Okayama 700-8558, Japan |
| |
Abstract: | BackgroundIdiopathic pulmonary fibrosis (IPF) is a chronically progressive lung disease with exceptionally poor prognosis. While lung transplantation (LTx) is considered the last-resort therapeutic option, dismal waitlist mortality still hampers the salvage of patients with IPF. Pirfenidone, originally designed for IPF treatment, has increasingly been utilized. This study aimed to evaluate whether Pirfenidone could influence outcomes of patients with IPF on the Japanese LTx waitlist.MethodsThis retrospective single-center cohort study included 25 consecutive patients with IPF who were registered as LTx candidates at our institution between July 1999 and August 2016. Patients with a history of pretransplant Pirfenidone therapy (Pirfenidone group) were compared with those with no history (non-Pirfenidone group).ResultsIn total, 6 (24%) patients received Pirfenidone as pretransplant therapy for 45.2 (range, 18.6–66.8) months. During the treatment period, the Pirfenidone group achieved a significant reduction in the decline rate of the forced vital capacity (-6.2% vs. -0.3%, p = 0.04) and a lower lung allocation score (31 vs. 41, p = 0.013) compared with the non-Pirfenidone group. The Pirfenidone group exhibited 100% waitlist survival three years after registration that was comparable to other indications, and 66% of the patients were still alive at the time of organ availability. No patient in the Pirfenidone group developed Pirfenidone-related surgical complications postoperatively.ConclusionsPatients with IPF successfully managed with long-term Pirfenidone therapy achieved favorable outcomes after LTx registration, comparable to other patients with LTx indications. The tolerability to antifibrotic therapy can be a predictor of waitlist survival. |
| |
Keywords: | IPF idiopathic pulmonary fibrosis LTx lung transplantation FVC forced vital capacity LAS lung allocation score ILD interstitial lung disease LDLLT living-donor lobar lung transplantation Idiopathic pulmonary fibrosis Pirfenidone Lung transplantation Bridge therapy |
本文献已被 ScienceDirect 等数据库收录! |
|