Idiopathic pulmonary fibrosis (IPF) is a progressive debilitating lung disease with considerable morbidity. Heterogeneity in epidemiologic studies means the full impact of the disease is unclear. A targeted literature search for population-based, observational studies reporting incidence and/or prevalence of IPF from January 2009 to April 2020 was conducted. Identified studies were aggregated by country. For countries with multiple publications, a weighted average was determined. Incidence and prevalence data were adjusted for between-study differences where possible. The final model included adjusted estimates of incidence and prevalence per 10,000 of the population with 95% confidence intervals. As prevalence estimates vary depending on the definitions used, estimates were based on a specific case definition of IPF. Overall, 22 studies covering 12 countries met the inclusion criteria, with 15 reporting incidence and 18 reporting prevalence estimates. The adjusted incidence estimates (per 10,000 of the population) ranged from 0.35 to 1.30 in Asia–Pacific countries, 0.09 to 0.49 in Europe, and 0.75 to 0.93 in North America. Unadjusted and adjusted incidence estimates were consistent. The adjusted prevalence estimates ranged from 0.57 to 4.51 in Asia–Pacific countries, 0.33 to 2.51 in Europe, and 2.40 to 2.98 in North America. South Korea had the highest incidence and prevalence estimates. When prevalence estimates were compared to country-specific rare disease thresholds, IPF met the definition of a rare disease in all countries except South Korea. There were notable geographic gaps for IPF epidemiologic data. Due to differences in study methodologies, there is worldwide variability in the reported incidence and prevalence of IPF. Based on the countries included in our analysis, we estimated the adjusted incidence and prevalence of IPF to be in the range of 0.09–1.30 and 0.33–4.51 per 10,000 persons, respectively. According to these prevalence estimates, IPF remains a rare disease. For consistency, future epidemiologic studies of IPF should take age, sex, smoking status, and the specificity of case definitions into consideration.

中文翻译：

---

特发性肺纤维化的全球发病率和流行率

特发性肺纤维化 (IPF) 是一种发病率相当高的进行性肺病。流行病学研究的异质性意味着该疾病的全面影响尚不清楚。对报告 2009 年 1 月至 2020 年 4 月 IPF 发病率和/或患病率的基于人群的观察性研究进行了有针对性的文献检索。已确定的研究按国家/地区汇总。对于拥有多种出版物的国家，确定了加权平均值。在可能的情况下，针对研究之间的差异对发生率和患病率数据进行了调整。最终模型包括调整后的每 10,000 人的发病率和患病率估计值，置信区间为 95%。由于流行率估计值因使用的定义而异，因此估计值基于 IPF 的特定病例定义。全面的，涵盖 12 个国家的 22 项研究符合纳入标准，其中 15 项报告发病率和 18 项报告流行率估计值。调整后的发病率估计值（每 10,000 人）在亚太国家为 0.35 至 1.30，在欧洲为 0.09 至 0.49，在北美为 0.75 至 0.93。未调整和调整的发生率估计是一致的。调整后的患病率估计值在亚太国家为 0.57 至 4.51，欧洲为 0.33 至 2.51，北美为 2.40 至 2.98。韩国的发病率和流行率估计最高。当将流行率估计值与特定国家的罕见病阈值进行比较时，IPF 在除韩国外的所有国家都符合罕见病的定义。IPF 流行病学数据存在显着的地域差异。由于研究方法的不同，世界范围内报告的 IPF 发病率和流行率存在差异。根据我们分析中包括的国家，我们估计调整后的 IPF 发病率和患病率分别在每 10,000 人 0.09-1.30 和 0.33-4.51 的范围内。根据这些流行率估计，IPF 仍然是一种罕见疾病。为保持一致性，未来 IPF 的流行病学研究应考虑年龄、性别、吸烟状况和病例定义的特殊性。