Objective: To compare the trends in age-standardised incidence and mortality from interstitial lung diseases (ILD) in the United Kingdom (UK) and the European Union (EU).

Design: Observational study using data obtained from the Global Burden of Disease Study.

Setting and Participants: Residents of the UK and of the twenty-seven EU countries.

Main outcome measures: ILD age-standardised incidence rates per 100 000 (ASIR), age-standardised death rates per 100 000 (ASDR), and mortality-to-incidence ratio (MIRs) are presented for males and females separately for each country, for the years 2001–2017. Trends were analysed using Joinpoint regression analysis.

Results: For men, in 2017, the median incidence of ILD was 7.22 (IQR 5.57–8.96) per 100 000 population. For women, in 2017, the median incidence of ILD was 4.34 (IQR 3.36–6.29) per 100 000 population. For men, in 2017, the median ASDR attributed to ILD was 2.04 (IQR 1.13–2.71) per 100 000 population. For women, the median ASDR in 2017 for ILD was 1.02 (0.68–1.37) per 100 000 population. There was an overall increase in ASDR during the observation period with a median change of +20.42% (IQR 5.44–31.40) for men and an increase of +15.44% (IQR −1.01–31.52) for women. Despite increases in mortality over the entire observation period, there were decreasing mortality trends in the majority of countries at the end of the observation period (75% for men and 86% for women).

Conclusion: Over the past two decades, there have been increases in the incidence and mortality of interstitial lung diseases in Europe. The most recent trends, however, demonstrate decreases in mortality from ILD in the majority of European countries for both men and women. These data support the ongoing improvements in the diagnosis and management of ILD.