Primary biliary cholangitis mortality in Europe: a temporal and spatial view across the period 2002-2022

Primary biliary cholangitis (PBC) is a rare autoimmune disease of multifactorial origin with an uneven incidence across countries. This study sought to identify temporal trends in PBC mortality and describe possible spatial patterns across Europe.

Data on PBC mortality (ICD-10 K74.3) and European populations by age group in 20 countries were sourced from the World Health Organization for the period 2002-2022. We calculated age-adjusted mortality rates (AAMR) for the period 2012-2022, both for individual countries and overall. Trends were assessed using Joinpoint regression analysis. To ascertain the geographical distribution, standardised mortality ratios (SMR) were calculated for the periods 2002-2011 and 2012-2022.

A total of 12,934 PBC deaths (81.65% women) were recorded during the period 2002-2022. Mortality showed a downward trend in most countries except for Hungary, Türkiye, Romania and Poland. Lithuania registered the highest AAMR, at 3.47 per 1,000,000 population (95% CI 2.86-4.19), followed by Hungary and the United Kingdom (UK). During the period 2002-2011, the risk of death in northern countries was twice as high as expected (Finland, Lithuania and the UK with SMRs of 2.09 to 2.14). The differences between northern and southern Europe persisted during 2012-2022, albeit with some variations.

PBC mortality is decreasing or remains stable in most European countries. Risk of death is higher in countries in the north and north-west. There is a need for further in-depth study of the causes of the geographical variability detected.