There is no debate that idiopathic pulmonary fibrosis (IPF) is a devastating disease that has a significant impact on patient morbidity and mortality. Patients with IPF experience symptoms throughout most of their disease, with disabling dyspnoea and cough that reduce quality of life. Most IPF patients eventually succumb to respiratory failure with a median survival of approximately 3 years from the time of diagnosis [1]. Two antifibrotic medications slow progression of IPF [2–5]; however, it has yet to be shown whether IPF mortality will change substantially with the availability of these therapies. There are likely multiple reasons that IPF-related mortality appears to be increasing in most European countries http://ow.ly/Maty30gRrDl

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特发性肺纤维化死亡率上升：事实还是谬误？

毫无疑问，特发性肺纤维化 (IPF) 是一种破坏性疾病，对患者的发病率和死亡率有重大影响。IPF 患者在整个疾病过程中都会出现症状，呼吸困难和咳嗽会降低生活质量。大多数 IPF 患者最终死于呼吸衰竭，自诊断之日起的中位生存期约为 3 年[1]。两种抗纤维化药物可减缓 IPF 的进展 [2-5]；然而，尚未证明 IPF 死亡率是否会随着这些疗法的可用性而发生显着变化。在大多数欧洲国家，IPF 相关死亡率似乎在增加，可能有多种原因 http://ow.ly/Maty30gRrDl