OBJECTIVES: Wilson’s disease (WD), or hepatolenticular degeneration, is a rare autosomal recessive disorder of defected copper metabolism in the liver. This study summarizes the available evidence on its economic burden.

METHODS: PubMed^® and Embase^® were systematically searched, in accordance with Preferred Reporting Items for Systemic Reviews and Meta-Analyses guidelines, by pairing relevant keywords to identify English language studies reporting on WD’s economic burden, published from 2013 to 2023. Electronic searches were supplemented by bibliographic searches; publications were screened by two independent reviewers.

RESULTS: Of 387 publications identified, 12 global studies were included. In the cohorts identified from Truven Health MarketScan Commercial Claims database, average monthly total healthcare costs and resource utilization was significantly higher for patients with WD than those with chronic liver disease without WD ($3,887 versus $1,979 and 3.35 versus 2.65, respectively). The annual cost of hospitalization identified from the National Inpatient Sample consistently increased over time, from $5.94 million in 2002 to $35.58 million in 2014. In South Korea, mean annual total direct medical costs per person with WD were $1,643. Liver transplantation was the major cost driver in Germany, accounting for 44.5% (€24,205) of total annual costs of WD-related hospitalization (€1,336,901) in 2017. The average length of stay (LOS) in hospital for patients with WD ranged from 3.8 days due to metabolic reasons to 19.3 days due to liver transplant. Mean LOS for patients with WD with cirrhosis was considerably higher than those without cirrhosis (difference: 1.7 days; p < 0.01).

CONCLUSIONS: WD is associated with a substantial economic burden. Liver transplantation and inpatient admissions were the key cost and resource use drivers; furthermore, cost and resource use increase with comorbid liver disease. Additional data are required to understand the overall impact of costs associated with the management of WD, with or without comorbidity, from a societal perspective.