Department of Pulmonary and Critical Care Medicine, Shengjing Hospital of China Medical University, Shenyang, PR China.
Department of Pulmonary and Critical Care Medicine, Institute of Respiratory Diseases, the First Affiliated Hospital of China Medical University, No.155 Nanjing North Street, Shenyang, Liaoning 110001, China.
Ther Adv Respir Dis. 2020 Jan-Dec;14:1753466620963015. doi: 10.1177/1753466620963015.
Pirfenidone is the first antifibrotic drug approved for the treatment of idiopathic pulmonary fibrosis (IPF) and it is used in the treatment of other interstitial pneumonias, such as unclassifiable interstitial lung disease (ILD) and connective tissue-related ILD. This study examined the efficacy of pirfenidone in patients with IPF and fibrotic idiopathic non-specific interstitial pneumonia (f-iNSIP).
In a retrospective real-life study, 67 IPF and 24 f-iNSIP patients were enrolled and classified into a pirfenidone group and a non-antifibrotic group. The level of forced vital capacity (FVC) and diffusing capacity of lung for carbon monoxide (DLco) at baseline, 6, 12 and 24 months were recorded. The level of KL-6 in serum was detected by chemiluminescence enzyme immunoassay (CLEIA). The prognosis and safety outcomes were collected from patients.
In IPF patients, pirfenidone decreased the mean change of FVC and DLco, and decreased the proportion of patients with a ⩾10% decline in FVC or a ⩾15% decline in DLco compared with the non-antifibrotic group. There was no difference in the mean change of FVC and DLco between smokers and non-smokers who received pirfenidone treatment. There was an improvement in progression-free survival, defined as the time to the first occurrence of acute exacerbation or death related to pulmonary fibrosis. Moreover, the ratio of patients who experienced acute exacerbation and death related to pulmonary fibrosis was lower in the pirfenidone group. There was no change in lung function and prognosis between the pirfenidone and non-antifibrotic groups in f-iNSIP patients. The KL-6 level slightly decreased after 1 year of pirfenidone treatment but not significantly. Gastrointestinal and skin-related adverse events were most common, and four patients ceased treatment due to the side effects.
Pirfenidone safely reduced disease progression by improving the lung function and progression-free survival in IPF patients, with acceptable side effects. The efficacy of pirfenidone on f-iNSIP was not significant, suggesting the need for further studies..
吡非尼酮是首个获批用于治疗特发性肺纤维化（IPF）的抗纤维化药物，也用于治疗其他间质性肺病，如未分类间质性肺病（ILD）和结缔组织相关 ILD。本研究考察了吡非尼酮在 IPF 和纤维化特发性非特异性间质性肺炎（f-iNSIP）患者中的疗效。
在一项回顾性真实世界研究中，纳入了 67 例 IPF 和 24 例 f-iNSIP 患者，并分为吡非尼酮组和非抗纤维化组。记录基线、6、12 和 24 个月时用力肺活量（FVC）和一氧化碳弥散量（DLco）的水平。采用化学发光酶免疫分析法（CLEIA）检测血清 KL-6 水平。从患者中收集预后和安全性结局。
在 IPF 患者中，吡非尼酮降低了 FVC 和 DLco 的平均变化值，并降低了 FVC 下降 ⩾10%或 DLco 下降 ⩾15%的患者比例，与非抗纤维化组相比。接受吡非尼酮治疗的吸烟者和不吸烟者的 FVC 和 DLco 平均变化值无差异。无进展生存期（定义为首次发生急性加重或与肺纤维化相关的死亡的时间）得到改善。此外，吡非尼酮组发生急性加重和与肺纤维化相关的死亡的患者比例较低。f-iNSIP 患者中，吡非尼酮组和非抗纤维化组的肺功能和预后均无变化。吡非尼酮治疗 1 年后 KL-6 水平略有下降，但无统计学意义。最常见的胃肠道和皮肤相关不良事件，有 4 例患者因副作用停止治疗。
吡非尼酮通过改善肺功能和无进展生存期，安全地减缓了 IPF 患者的疾病进展，具有可接受的副作用。吡非尼酮对 f-iNSIP 的疗效不显著，表明需要进一步研究。
