Krauss Ekaterina, Tello Silke, Wilhelm Jochen, Schmidt Johanna, Stoehr Mark, Seeger Werner, Dartsch Ruth C, Crestani Bruno, Guenther Andreas
European IPF Registry & Biobank (eurIPFreg/bank), 35392 Giessen, Germany.
Department of Medicine II, Universities of Giessen and Marburg Lung Center (UGMLC), Member of the German Center for Lung Research (DZL), 35392 Giessen, Germany.
J Clin Med. 2020 Nov 22;9(11):3763. doi: 10.3390/jcm9113763.
Idiopathic pulmonary fibrosis (IPF) is a chronic progressive fibrotic pulmonary disease with rising incidence. In this study the effectiveness of pirfenidone, as measured by longitudinal change in individual slope of forced vital capacity (FVC) prior to and after initiating pirfenidone treatment, was evaluated in IPF patients recruited into the European registry for idiopathic pulmonary fibrosis (eurIPFreg). Secondary variables were the evaluation of the change in individual slope of diffusion capacity of the lungs for carbon monoxide (DLco), the Borg dyspnea scale, and six-minute walking distance (6MWD), as well as survival analyses.
Data of 122 eurIPFreg patients, who had at least two pulmonary function tests (PFTs) prior to or under treatment with pirfenidone, were analyzed by calculating slope-changes. The global analysis revealed an average slope change of +1.48 ± 0.28 (% per annum (p.a)) after start of treatment ( < 0.001), reflecting a reduction in annual FVC decline of approx. 50% under pirfenidone; it also showed a reduction in DLco, and increase in 6MWD (both < 0.0001), as well as a flattening of the Borg dyspnea scale ( = 0.02). The median survival under treatment was 4.82 years. Patients with a more restrictive disease (FVC < 80% pred.), with a rapid progression (FVC decline >10% pred. p.a.), previous smokers and patients > 60 years of age seemed to profit more from pirfenidone treatment.
We report the effectiveness of pirfenidone in a European "real world" IPF cohort with outcome data extending up to 9 years. Global analyses demonstrated a positive effect of pirfenidone on the decline of the lung function over time. Survival was dependent on Gender-Age-Physiology (GAP) score and age prior to therapy.
特发性肺纤维化（IPF）是一种发病率不断上升的慢性进行性纤维化肺部疾病。在本研究中，通过在开始使用吡非尼酮治疗前后用力肺活量（FVC）个体斜率的纵向变化来衡量吡非尼酮的有效性，对纳入欧洲特发性肺纤维化注册研究（eurIPFreg）的IPF患者进行了评估。次要变量包括对肺一氧化碳弥散量（DLco）个体斜率变化、Borg呼吸困难量表和六分钟步行距离（6MWD）的评估，以及生存分析。
对122例在使用吡非尼酮治疗前或治疗期间至少进行过两次肺功能测试（PFT）的eurIPFreg患者的数据进行斜率变化计算分析。整体分析显示，治疗开始后平均斜率变化为+1.48±0.28（每年百分比（p.a））（<0.001），这反映在吡非尼酮治疗下每年FVC下降约50%；还显示DLco下降，6MWD增加（均<0.0001），以及Borg呼吸困难量表变平缓（=0.02）。治疗期间的中位生存期为4.82年。病情限制更严重（FVC<预测值的80%）、进展迅速（FVC下降>预测值的10%每年）、既往吸烟者和60岁以上患者似乎从吡非尼酮治疗中获益更多。
我们报告了吡非尼酮在欧洲“真实世界”IPF队列中的有效性，结果数据长达9年。整体分析表明吡非尼酮对肺功能随时间下降有积极作用。生存取决于性别 - 年龄 - 生理学（GAP）评分和治疗前年龄。
