Population‐Based Incidence, Prevalence and Survival of Systemic Light Chain Amyloidosis in Sweden

Objectives There are few population‐based epidemiological studies in light chain (AL) amyloidosis. We aimed to determine the incidence, prevalence and survival of AL amyloidosis in the general population of defined geographic regions of Sweden and to analyse trends over time. Methods We identified all new cases of systemic AL amyloidosis from Uppsala and Stockholm County of Sweden with a biopsy‐verified and confirmed diagnosis during 2000–2020. Results 302 cases were identified, 76 from Uppsala and 226 from Stockholm County. The age‐ and sex‐adjusted incidence rate was 12.0 (95% CI: 9.3–14.7) per million person‐years for Uppsala County, where the identification of cases was complete. There was no significant change in the incidence over time (test for trend p = 0.153). The 5‐year limited duration prevalence increased from 9.9 cases per million in 2004 to 38.6 in 2020, and the trend was not statistically significant ( p = 0.434). Median survival for all patients was 21 months (95% CI: 14.5–27.5) and increased significantly from 13 to 28 months ( p < 0.001) during the period. Conclusions We report the population‐based incidence, prevalence and survival of AL amyloidosis in Sweden and observed an increased survival but no significant change in the incidence or prevalence over time.