Idiopathic pulmonary fibrosis (IPF) is a progressive lung disease caused by a combination of genetic and environmental factors [1]. Studies indicate that the global incidence and prevalence of IPF are increasing [2–8]. The reasons for this are not fully understood, but contributors include more widespread use of computed tomography (CT) imaging, improved recognition of the disease, an ageing population, and greater exposure to environmental pollutants and other risk factors. Mortality rates in patients with well-defined IPF participating in multicentre clinical trials in the modern treatment era, which began in the early 2000s, have decreased (figure 1). Prior to that, the standard of care for IPF consisted of treatment with corticosteroids, azathioprine and other immunosuppressive medications, which have been shown to be associated with higher mortality [9–13].

特发性肺纤维化(IPF)是一种由遗传和环境因素共同引起的进行性肺部疾病[1]。研究表明，IPF 的全球发病率和患病率正在增加 [2-8]。其原因尚不完全清楚，但影响因素包括计算机断层扫描 (CT) 成像的更广泛使用、对疾病的认识提高、人口老龄化以及更多地接触环境污染物和其他风险因素。在 2000 年代初开始的现代治疗时代，参加多中心临床试验的明确 IPF 患者的死亡率有所下降（图 1）。在此之前，IPF 的护理标准包括皮质类固醇、硫唑嘌呤和其他免疫抑制药物的治疗，这些药物已被证明与较高的死亡率相关[9-13]。