Mortality associated with Behçet’s disease in France assessed by multiple-cause-of-death analysis

Objectives To examine the mortality rates and causes of death among French decedents with Behçet’s disease (BD). Methods Data collected between 1979 and 2016 by the French Epidemiological Center for the Medical Causes of Death was used to calculate BD-related mortality rates and examine differences according to age and sex, determine underlying and non-underlying causes of death (UCD/NUCD), and compare with the general population (observed/expected ratios, O / E ). Results A total of 328 death certificates mentioned BD as UCD ( n = 171) or NUCD ( n = 157). The mean (± SD ) age at death was 52.8 (± 11.3) years in men and 58.4 (± 16.5) in women (vs. 71.2 and 79.8 in the general population, respectively). The age-standardized mortality rate was 0.15/million person-years and significantly increased over the study period ( p < 0.01). When BD was the UCD, the most frequent associated causes were infections (25.1%), underlying organ dysfunction (21.6%), and arterial events (15.2%). As compared to the general population, BD decedents < 45 years were more likely to die from a cardiovascular event ( O / E = 6.25, p < 0.001); decedents > 45 years were more likely to die from infection ( O / E = 9.69, p < 0.001). Between 1979 and 2016, BD as UCD decreases whereas NUCD increased. Conclusion This study found an earlier mean age at death for BD compared to the general population; young patients were more prone to die from cardiovascular complication whereas older patients died more frequently from infections. Over the years, the mean age at death increased and BD was less frequently considered as an UCD, suggesting a better knowledge of the disease and complications. Key Points • Patients with Behçet’s disease die earlier than the general population. • Young patients were more prone to die from cardiovascular complication. • Older patients died more frequently from infections.