Tanaka Shin, Miyoshi Kentaroh, Higo Hisao, Kurosaki Takeshi, Otani Shinji, Sugimoto Seiichiro, Yamane Masaomi, Kiura Katsuyuki, Toyooka Shinichi, Oto Takahiro
Department of Thoracic Surgery, Okayama University Hospital, 2-5-1, Shikata-cho, Kita-ku, Okayama 700-8558, Japan.
Department of Thoracic Surgery, Okayama University Hospital, 2-5-1, Shikata-cho, Kita-ku, Okayama 700-8558, Japan; Department of Thoracic Surgery, Okayama Medical Center, 1711-1 Tamasu, Kita-ku, Okayama 701-1192, Japan.
Respir Investig. 2019 Mar;57(2):165-171. doi: 10.1016/j.resinv.2018.12.002. Epub 2018 Dec 30.
Idiopathic 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.
This 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).
In 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.
Patients 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.
特发性肺纤维化（IPF）是一种慢性进行性肺部疾病，预后极差。虽然肺移植（LTx）被认为是最后的治疗选择，但等待名单上的死亡率很高，这仍然阻碍了IPF患者的救治。吡非尼酮最初用于治疗IPF，目前其应用越来越广泛。本研究旨在评估吡非尼酮是否会影响日本LTx等待名单上IPF患者的预后。
这项回顾性单中心队列研究纳入了1999年7月至2016年8月期间在本机构登记为LTx候选者的25例连续IPF患者。将有移植前吡非尼酮治疗史的患者（吡非尼酮组）与无该治疗史的患者（非吡非尼酮组）进行比较。
共有6例（24%）患者接受了吡非尼酮作为移植前治疗，疗程为45.2（范围18.6 - 66.8）个月。在治疗期间，与非吡非尼酮组相比，吡非尼酮组的用力肺活量下降率显著降低（-6.2%对-0.3%，p = 0.04），肺分配评分更低（31对41，p = 0.013）。吡非尼酮组在登记三年后的等待名单生存率为100%，与其他适应证相当，66%的患者在器官可用时仍然存活。吡非尼酮组术后无患者发生与吡非尼酮相关的手术并发症。
长期接受吡非尼酮治疗且病情得到成功控制的IPF患者，在LTx登记后的预后良好，与其他有LTx适应证的患者相当。抗纤维化治疗的耐受性可能是等待名单生存率的一个预测指标。
