Wilson's Disease in Finland: A Nationwide Population‐Based Study

Abstract

Background <div>Data on the epidemiology and prognosis of Wilson's disease are scarce, and no clinical data are available from Finland. Methods </div><div>All persons diagnosed and treated for Wilson's disease in Finnish hospitals in 1998 to 2017 were identified. Data were collected from national registries and patient charts. </div><div>Results </div><div>The point prevalence was 0.45/100,000 (95% confidence interval, 0.29-0.67) on December 31, 2017, but no more than 0.35/100,000 (95% confidence interval, 0.21-0.55) among native Finns. Annual incidence was 0.016/100,000 (95% confidence interval, 0.0093-0.026). Median age at diagnosis was 15.8 years (interquartile range, 8.3-32.2; range, 3.8-48.1 years). Upon presentation, liver damage was observed in 58%, neurological signs and symptoms (most often tremor and dysarthria) in 40%, and 32% of patients were asymptomatic. Patients had poorer long-term survival (hazard ratio, 2.92 for death;P= 0.005) compared with matched controls. </div><div>Conclusions </div><div>Wilson's disease is very rare in Finland. Patients have an increased risk of death indicating an unmet treatment need. </div>