Abstract
Mortality from idiopathic pulmonary fibrosis (IPF) is increasing in most European countries, but there are no data for Italy. We analysed the registry data from a region in northeastern Italy to assess the trends in IPF-related mortality during 2008–2019, to compare results of underlying vs. multiple cause of death analyses, and to describe the impact of the COVID-19 epidemic in 2020. We identified IPF (ICD-10 code J84.1) among the causes of death registered in 557,932 certificates in the Veneto region. We assessed time trends in annual age-standardized mortality rates by gender and age (40–74, 75–84, and ≥85 years). IPF was the underlying cause of 1310 deaths in the 2251 certificates mentioning IPF. For all age groups combined, the age-standardized mortality rate from IPF identified as the underlying cause of death was close to the European median (males and females: 3.1 and 1.3 per 100,000/year, respectively). During 2008–2019, mortality rates increased in men aged ≥85 years (annual percent change of 6.5%, 95% CI: 2.0, 11.2%), but not among women or for the younger age groups. A 72% excess of IPF-related deaths was registered in March–April 2020 (mortality ratio 1.72, 95% CI: 1.29, 2.24). IPF mortality was increasing among older men in northeastern Italy. The burden of IPF was heavier than assessed by routine statistics, since less than two out of three IPF-related deaths were directly attributed to this condition. COVID-19 was accompanied by a remarkable increase in IPF-related mortality.
Highlights
Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, and fibrotic lung disease of unknown causes, occurring primarily in older adults [1]
For both the underlying cause of death (UCOD) and the Multiple cause of death (MCOD) analyses, IPFrelated mortality rates were higher in males compared to females, and they increased steeply with age
Our study showed an association between this condition and IPF (OR: 2.9, 95% confidence intervals (95% CI): 1.3, 6.5), despite the fact that esophagitis or reflux disease were only reported in 0.3% of death certificates with a mention of IPF
Summary
Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, and fibrotic lung disease of unknown causes, occurring primarily in older adults [1]. It is the most common idiopathic interstitial pneumonia and holds the worst prognosis [2]. Evidence suggests that the incidence and prevalence of IPF range between 2–30 and 10–60 per 100,000/year, respectively, but comparisons across studies are difficult owing to differences in data sources and case definitions [3]. According to the World Health Organization mortality database, the median age-standardized mortality rates from IPF in European countries in 2001–2013 were 3.75 per 100,000 and 1.50 per 100,000 for males and females, respectively [7]. No data on mortality are available for Italy
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