Department of Medical and Surgical Sciences and Neurosciences, Respiratory Disease and Lung Transplant Unit, University of Siena, AOUS, Siena, Italy.
Department of Medical and Surgical Sciences and Neurosciences, Respiratory Disease and Lung Transplant Unit, University of Siena, Viale Bracci, Siena, 53100, Italy.
Ther Adv Respir Dis. 2020 Jan-Dec;14:1753466620906326. doi: 10.1177/1753466620906326.
Idiopathic pulmonary fibrosis (IPF) is the most common idiopathic interstitial pneumonia and has a median survival after diagnosis of 2-5 years. Pirfenidone is the first approved antifibrotic drug for the treatment of IPF. Here we report the functional progress, side effects and survival data of a population of patients with IPF, diagnosed at our centre and treated with pirfenidone.
We enrolled 91 patients with IPF (71 males) treated with pirfenidone. Clinical, survival and functional details were collected retrospectively at start of therapy and after 12, 24, 36 and 48 months of treatment. Lung function tests at least 12 months before starting therapy were available for 40 patients and were entered in the database, as well as side effects.
During the observation period (922 ± 529 days), 27 patients died, 5 patients underwent lung transplant and 10 patients interrupted therapy due to adverse events or IPF progression. The median survival was 1606 days. There was a significant reduction in disease progression rate, as measured by trend of forced vital capacity, after 1 year of therapy with respect to before treatment ( = 0.0085). Forced vital capacity reduction rate was progressively higher in the subsequent years of treatment. Treatment-related side effects were reported in 25 patients and were predominantly mild. Overall, four patients discontinued therapy due to severe photosensitivity.
Our findings confirm the efficacy of pirfenidone in reducing functional progression of IPF and its excellent safety profile in a real-life setting. This study, designed on a long-term follow up, contributes to the growing evidence on safety, tolerability and efficacy of pirfenidone in IPF.
特发性肺纤维化（IPF）是最常见的特发性间质性肺炎，诊断后中位生存期为 2-5 年。吡非尼酮是首个获批用于治疗 IPF 的抗纤维化药物。在此，我们报告了在我们中心诊断并接受吡非尼酮治疗的 IPF 患者的功能进展、副作用和生存数据。
我们纳入了 91 例接受吡非尼酮治疗的 IPF 患者（71 例男性）。在开始治疗时以及治疗 12、24、36 和 48 个月后，回顾性收集了临床、生存和功能详细信息。40 例患者在开始治疗前至少 12 个月进行了肺功能检查，并将其纳入数据库，同时记录了副作用。
在观察期间（922±529 天），27 例患者死亡，5 例患者接受了肺移植，10 例患者因不良反应或 IPF 进展而中断治疗。中位生存期为 1606 天。与治疗前相比，治疗 1 年后，用力肺活量的趋势表明疾病进展率显著降低（ = 0.0085）。在随后的治疗年份中，用力肺活量下降率逐渐升高。25 例患者报告了与治疗相关的副作用，主要为轻度。总体而言，有 4 例患者因严重光过敏而停止治疗。
我们的研究结果证实了吡非尼酮在降低 IPF 功能进展方面的疗效，以及在真实环境中的良好安全性。这项长期随访设计的研究为吡非尼酮在 IPF 中的安全性、耐受性和疗效提供了更多的证据。
