Background Owing to discrepancies in methodologies and how idiopathic pulmonary fibrosis (IPF) is diagnosed it is challenging to establish a consistent understanding of the disease burden In the UK, over 10 years ago, the incidence and prevalence of IPF were reported as 2.8–8.7 per 100 000 person-years (from 2000 to 2012) and 39 per 100 000 persons (in 2012), respectively. Here, we estimated the incidence and prevalence of IPF in England from 2008 to 2018 and investigated IPF mortality. Methods Using Clinical Practice Research Datalink Aurum and Hospital Episode Statistics (HES) linked datasets, we estimated incidence and prevalence using four validated diagnostic-code-based algorithms. Using the registered number of deaths (from Office of National Statistics) with the underlying cause being recorded as IPF, we estimated IPF mortality for the same period. Results Using Aurum-based definitions, incidence increased over time by 100% for Aurum narrow (3–6.1 per 100 000 person-years) and by 25% for Aurum broad (22.4–28.6 per 100 000 person-years). However, using HES-based definitions showed a decrease in incidence over the same period and lay between the two extremes derived for Aurum-based definition. IPF mortality in 2018 was 7.9 per 100 000 person-years and increased by 53% between 2008 and 2018. Interpretation When using best-case definitions, incidence rose throughout the study period. Scaling this to England’s population (2018), our best estimate would be in the range of 8000–9000 new cases per year which is higher than previously reported estimates (5000–6000). This increased burden in the new cases of IPF each year impacts future health service planning and resource allocation. Data may be obtained from a third party and are not publicly available. Data are available on request from the CPRD. Their provision requires the purchase of a license, and this license does not permit the authors to make them publicly available to all. To allow identical data to be obtained by others, via the purchase of a license, the code lists will be provided upon request. Licenses are available from the CPRD ([http://www.cprd.com][1]): The Clinical Practice Research Datalink Group, The Medicines and Healthcare products Regulatory Agency, 10 South Colonnade, Canary Wharf, London E14 4PU. [1]: http://www.cprd.com/

中文翻译：

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2008年至2018年英国特发性肺纤维化的发病率、患病率和死亡率：一项队列研究


背景 由于方法学和特发性肺纤维化 (IPF) 诊断方式的差异，对疾病负担建立一致的理解具有挑战性。在英国，10 多年前，IPF 的发病率和患病率据报道为 2.8-8.7%。分别为 10 万人年（2000 年至 2012 年）和每 10 万人 39 人（2012 年）。在这里，我们估计了 2008 年至 2018 年英国 IPF 的发病率和患病率，并调查了 IPF 死亡率。方法 使用临床实践研究数据链 Aurum 和医院发作统计 (HES) 链接数据集，我们使用四种经过验证的基于诊断代码的算法估计发病率和患病率。利用登记的死亡人数（来自国家统计局）以及记录为 IPF 的根本原因，我们估计了同期的 IPF 死亡率。结果 使用基于 Aurum 的定义，随着时间的推移，Aurum 狭窄的发病率增加了 100%（每 10 万人年 3-6.1 例），Aurum 广泛的发病率增加 25%（每 10 万人年 22.4-28.6 例）。然而，使用基于 HES 的定义显示同期发病率有所下降，并且介于基于 Aurum 的定义得出的两个极端之间。 2018 年 IPF 死亡率为每 10 万人年 7.9 例，2008 年至 2018 年间增加了 53%。 解释 当使用最佳案例定义时，发病率在整个研究期间呈上升趋势。如果将其扩展到英格兰人口（2018 年），我们的最佳估计是每年新增病例 8000-9000 例，高于之前报告的估计值（5000-6000 例）。每年新增特发性肺纤维化病例造成的负担增加，会影响未来的卫生服务规划和资源分配。数据可能从第三方获得，并且不公开。 数据可根据 CPRD 的要求提供。它们的提供需要购买许可证，并且该许可证不允许作者将它们公开提供给所有人。为了允许其他人通过购买许可证获得相同的数据，将根据要求提供代码列表。许可证可从 CPRD ([http://www.cprd.com][1]) 获取：临床实践研究数据链组、药品和保健产品监管机构、10 South Colonnade、Canary Wharf、London E14 4PU。 [1]：http://www.cprd。com/