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加拿大艾伯塔省间质性肺疾病的发病率、患病率和死亡率:一项基于人群的研究。

Incidence, Prevalence, and Mortality of Interstitial Lung Diseases in Alberta, Canada: A Population-based Study.

作者信息

Johannson Kerri A, Liu Zhaoyu, Southern Danielle A, Kalluri Meena, Gershon Andrea S, Farrand Erica, Grant-Orser Amanda, Fisher Jolene H

机构信息

Department of Medicine.

Department of Community Health Sciences.

出版信息

Ann Am Thorac Soc. 2025 Jul;22(7):1009-1017. doi: 10.1513/AnnalsATS.202406-625OC.


DOI:10.1513/AnnalsATS.202406-625OC
PMID:40068156
Abstract

The epidemiology of adult interstitial lung disease (ILD) is uncertain, given heterogeneous estimates from prior studies. We sought to define the incidence, prevalence, and mortality of ILD over a 10-year period using population-based data. We created an administrative ILD cohort in Alberta, Canada between 2010 and 2019 using population-based administrative data (inpatient, ambulatory, and outpatient physician billing databases) for a repeat cross-sectional study. Case definitions were developed from an established ILD cohort and applied to the general population, with performance characteristics tested using a nested case-control design. Age- and sex-standardized annual incidence and point prevalence rates were estimated for ILD overall and within diagnostic subgroups, with trends over time, per 100,000 at-risk adults and to permit comparisons with other studies, per 100,000 total population. Cox models estimated risk of death or lung transplantation. Between 2010 and 2019, 31,492 incident and 42,549 prevalent adult ILD cases were identified. The case definition for ILD performed well with 96.8% sensitivity, 98.5% specificity, and a positive predictive value of 94.3% in the population cohort. Mean age-standardized ILD incidence was 107.9/100,000 at-risk adults, 90.9/100,000 for females and 129.1/100,000 for males. Age-standardized ILD point prevalence increased from 416.5/100,000 at-risk adults in 2010 to 789.7/100,000 in 2019, higher in males versus females, and in rural versus urban areas. Age-standardized mean idiopathic pulmonary fibrosis (IPF) incidence was 36.9/100,000 at-risk adults, and point prevalence was 205.3/100,000 at-risk adults in 2019. Mean age-standardized ILD incidence and prevalence was 84 and 516.9/100,000 total population, respectively. One-year all-cause mortality for ILD patients decreased from 14.5% in 2011 to 11.7% in 2018 (for 2018 vs. 2011, adjusted rate ratio = 0.76; 95% confidence interval = 0.67-0.86). One-year all-cause mortality for IPF similarly decreased from 20.9% in 2011 to 14.7% in 2018 (for 2018 vs. 2011, adjusted rate ratio = 0.71; 95% confidence interval = 0.59-0.85), representing improved survival. In this population-based cohort, claims-based case definitions derived from an established ILD cohort performed well to develop an administrative cohort. Incidence remained stable over time, whereas prevalence increased and mortality decreased, for ILD overall and within the IPF subgroup. These estimates are higher than most prior reports, suggesting an overall underestimate of ILD burden.

摘要

鉴于先前研究的估计结果存在差异,成人间质性肺疾病(ILD)的流行病学情况尚不明确。我们试图利用基于人群的数据来确定10年间ILD的发病率、患病率和死亡率。我们在2010年至2019年期间,利用基于人群的行政数据(住院、门诊和门诊医生计费数据库)在加拿大艾伯塔省创建了一个行政ILD队列,用于重复横断面研究。病例定义是根据一个已建立的ILD队列制定的,并应用于一般人群,其性能特征通过巢式病例对照设计进行测试。对总体ILD以及诊断亚组内的年龄和性别标准化年发病率和点患病率进行了估计,并分析了随时间的趋势,以每10万名有风险的成年人计算,同时为便于与其他研究进行比较,也以每10万总人口计算。Cox模型估计了死亡或肺移植的风险。在2010年至2019年期间,共识别出31492例新发成人ILD病例和42549例现患成人ILD病例。ILD的病例定义在人群队列中表现良好,敏感性为96.8%,特异性为98.5%,阳性预测值为94.3%。年龄标准化的ILD平均发病率为每10万名有风险的成年人中107.9例,女性为90.9例/10万,男性为129.1例/10万。年龄标准化的ILD点患病率从2010年每10万名有风险的成年人中的416.5例增加到2019年的789.7例,男性高于女性,农村地区高于城市地区。年龄标准化的特发性肺纤维化(IPF)平均发病率为每10万名有风险的成年人中36.9例,2019年的点患病率为每10万名有风险的成年人中205.3例。年龄标准化的ILD平均发病率和患病率分别为每10万总人口中84例和516.9例。ILD患者的一年全因死亡率从2011年的14.5%降至2018年的11.7%(2018年与2011年相比,调整后的率比=0.76;95%置信区间=0.67 - 0.86)。IPF的一年全因死亡率同样从2011年的20.9%降至2018年的14.7%(2018年与2011年相比,调整后的率比=0.71;95%置信区间=0.59 - 0.85),这表明生存率有所提高。在这个基于人群的队列中,源自已建立的ILD队列的基于索赔的病例定义在构建行政队列方面表现良好。总体ILD以及IPF亚组内的发病率随时间保持稳定,而患病率上升,死亡率下降。这些估计值高于大多数先前的报告,表明ILD负担总体上被低估了。

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