Regional Referral Centre for Rare Lung Diseases, A.O.U. Policlinico-Vittorio Emanuele, University of Catania, Catania, Italy.
Regional Referral Centre for Rare Lung Diseases, A.O.U. Policlinico-Vittorio Emanuele, University of Catania, Catania, Italy
Eur Respir Rev. 2017 Oct 3;26(145). doi: 10.1183/16000617.0053-2017. Print 2017 Sep 30.
Idiopathic pulmonary fibrosis (IPF) is characterised by progressive changes of the lung architecture causing cough and dyspnoea and ultimately leading to lung failure and death. Today, for the first time, two drugs that may reduce the inexorable progression of the disease are available, suggesting that treatment with specific drugs for IPF should be started as soon as diagnosis is made. This applies to any disease and particularly to IPF, which is marked by a 5-year survival comparable or even worse than many cancers. However, despite common sense and even worse, in spite of scientific data coming from clinical trials, analysis, long-term safety studies and real-life experiences, the question of when to start and when to stop treatment with antifibrotics is still debated. In IPF, particularly when the disease is diagnosed at an early stage, "wait and watch" behaviour is not rare to observe. This is largely due to the lack of awareness of both patients and clinicians regarding the progression of the disease and its prognosis. Another important issue is when treatment should be stopped. In general, there are two main reasons to stop a therapy: unbearable side-effects and/or lack of efficacy. According to current (although preliminary) evidence, antifibrotic drugs should not be discontinued except for safety issues.
特发性肺纤维化(IPF)的特征是肺结构的进行性改变,导致咳嗽和呼吸困难,最终导致肺衰竭和死亡。如今,首次有两种可能减缓疾病不可避免进展的药物可用,这表明一旦确诊,就应开始使用针对 IPF 的特定药物治疗。这适用于任何疾病,尤其是特发性肺纤维化,其 5 年生存率与许多癌症相当,甚至更差。然而,尽管有常识,甚至更糟糕的是,尽管有来自临床试验、分析、长期安全性研究和实际经验的科学数据,何时开始和停止使用抗纤维化药物的问题仍在争论中。在特发性肺纤维化中,特别是在疾病早期诊断时,观察到“等待和观察”的行为并不罕见。这主要是由于患者和临床医生对疾病进展及其预后缺乏认识。另一个重要问题是何时停止治疗。一般来说,停止治疗有两个主要原因:无法忍受的副作用和/或缺乏疗效。根据目前(尽管初步)的证据,除了安全问题外,不应停止抗纤维化药物治疗。