Mortality trends in idiopathic pulmonary fibrosis in Europe between 2013 and 2018

Abstract

Background: Previous research has suggested that the incidence of idiopathic pulmonary fibrosis (IPF) is increasing in the UK and elsewhere. The aim of this study is to provide contemporary estimates of IPF mortality rates across 24 European Union (EU) countries from 2013 to 2018, using death certificate data from the European Statistics Institution (EUROSTAT) database.

Methods: We extracted country data for IPF (International Classification of Diseases, 10th Revision: code J84.1) mortality from the EUROSTAT dataset. We calculated country-, age- and sex-specific death registration rates between 2013 and 2018. We used direct standardisation to compare rates between countries. We calculated annual trends in mortality rate ratios using a segmented regression model.

Results: The overall standardised mortality rate in 24 EU countries during this period was 3.90 (95% CI 3.80-3.90) per 100 000 person-years, with the rate rising from 3.70 in 2013 to 4.00 in 2018 (average annual percent change 1.74%, 95% CI 0.91-2.59%). We observed substantial inter-country differences, with the highest rates detected in Ireland, the UK and Finland, the lowest rate in Bulgaria, and middle rates in Germany, Greece, Italy, the Netherlands, Portugal and Slovenia.

Conclusions: The IPF mortality rate is increasing across Europe. There are currently more than 17 000 deaths recorded from IPF each year in Europe but the marked geographical differences we observed suggest that this figure may underestimate the true rate considerably.

Overview of the study. IPF: idiopathic pulmonary fibrosis; IPF-CS: idiopathic pulmonary fibrosis-clinical syndrome; EU: European Union.

FIGURE 1

Geographical distribution of idiopathic pulmonary fibrosis-clinical syndrome average age- and sex-standardised mortality rates in 24 European Union countries in 2015–2017. Mortality rates reported as number of deaths per 100 000 person-years.

FIGURE 2

a) Average age- and sex-standardised mortality rates (95% CI) of idiopathic pulmonary fibrosis-clinical syndrome (IPF-CS) in 24 European Union (EU) countries in 2015–2017. b) Average percent annual change (AAPC) in IPF-CS mortality rate in 24 EU countries in 2013–2018.

FIGURE 3

Geographical distribution of idiopathic pulmonary fibrosis-clinical syndrome mortality rate trends in 24 European Union countries from 2013 to 2018. Upward-pointing double arrows (↑↑) and a single upward arrow (↑) indicate overall increasing trends. Downward-pointing double arrows (↓↓) and a single downward arrow (↓) signify overall decreasing trends. A horizontal arrow (→) denotes stability in trends. *: statistically significant variations (p<0.05).