PRIMARY IMMUNODEFICIENCY DISEASE MORTALITY TRENDS ACROSS EU15+ COUNTRIES: AN EPIDEMIOLOGICAL ANALYSIS

Abstract

<h3>Introduction</h3> Primary Immunodeficiency Diseases (PIDDs) are rare genetic conditions that affect the immune system and are associated with high mortality. While the prevalence has been increasing there is a paucity of data on mortality trends from PIDD. The current investigation was to assess the burden of adult PIDD across EU15+ countries. <h3>Methods</h3> We extracted crude mortality data for EU15+ countries from the World Health Organization (WHO) Mortality Database according to the International Classification of Disease 10th Edition codes. We computed adult (age > 20) mortality rates per 1,000,000 population using the World Standard Population between 2000 and 2019. We report current mortality rates per 1,000,000 population as well as percentage change in mortality rates between EU15+ countries. <h3>Results</h3> Data were available for 15 countries for both men and women. The median ASMR for all countries in 2000 was 0.78 and 0.36 for men and women, respectively. By 2019, the median ASMR for men and women was 0.81 and 0.53, respectively. The highest mortality rates in 2019 for men was in Belgium (1.65) and for women in Denmark (1.28). There were 10 (67%) of countries with increasing mortality from PIDD for both men and women. The greatest increases in mortality for men was in Norway (+781%) and for women in Denmark (+258%). <h3>Conclusion</h3> We report significant variation in the burden of PIDD between EU15+ countries. Although the overall mortality rates remain low overall, there are significant increases in PIDD mortality across the majority of countries for both men and women.