Moua Teng, Ryu Jay H
Department of Medicine, Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester MN, USA,
Ther Clin Risk Manag. 2019 Jan 3;15:73-81. doi: 10.2147/TCRM.S160248. eCollection 2019.
Idiopathic pulmonary fibrosis is a progressive and fatal fibrotic lung disease which has seen new opportunity for drug treatment in the last several years with the approval of nintedanib and pirfenidone, two antifibrotic agents aimed at slowing decline in lung function as defined by FVC on pulmonary function testing. Despite these promising effects, delays in drug initiation have been reported undermining the premise that earlier drug initiation may sustain lung function and prolong survival. This review explores obstacles to earlier treatment, inclusive of defining so-called early idiopathic pulmonary fibrosis, difficulties in achieving a confident diagnosis in that setting, and uncertainties regarding drug-related benefits among specific patient subgroups such as those with no symptoms or advanced disease at presentation. Goals of therapy balanced with the burdens associated with antifibrotic drug therapy are negotiated on an individual basis. We review the evidence for and against earlier initiation of antifibrotic drug therapy along with its role in patient-centered outcomes.
特发性肺纤维化是一种进行性且致命的纤维化肺部疾病，在过去几年中，随着两种抗纤维化药物尼达尼布和吡非尼酮的获批，出现了新的药物治疗机会，这两种药物旨在减缓肺功能测试中由用力肺活量（FVC）定义的肺功能下降。尽管有这些令人鼓舞的效果，但据报道，药物起始延迟破坏了早期用药可能维持肺功能并延长生存期这一前提。本综述探讨了早期治疗的障碍，包括定义所谓的早期特发性肺纤维化、在此背景下做出可靠诊断的困难，以及特定患者亚组（如就诊时无症状或疾病晚期患者）中药物相关益处的不确定性。治疗目标需与抗纤维化药物治疗相关负担相权衡，这要因人而异。我们回顾了支持和反对早期启动抗纤维化药物治疗的证据及其在以患者为中心的结局中的作用。
